Castleman’s tumour (Castleman disease) is a rare disorder of lymph nodes. Lymph nodes are small glands that filter germs and help the immune system. In this disease, one or many lymph nodes grow too large and show special changes under the microscope. The immune system also becomes very active and can release many inflammatory chemicals called cytokines, especially interleukin-6 (IL-6). This can cause fever, weight loss, tiredness, night sweats, and problems in organs such as the liver, kidneys, and bone marrow.Wikipedia+2NCBI+2
Castleman’s tumour, also called Castleman disease, is a rare disorder where lymph nodes grow abnormally and make too many inflammatory proteins such as interleukin-6 (IL-6). This can be unicentric (one area of lymph nodes, often cured with surgery) or multicentric (many lymph node areas, often with whole-body symptoms like fever, weight loss, anaemia, and organ damage). Evidence-based guidelines show that surgery is the main treatment for unicentric disease, while biologic drugs such as siltuximab, rituximab, tocilizumab, and chemotherapy are used for multicentric disease.
Castleman’s tumour, also called Castleman disease, is a rare disorder where lymph nodes grow abnormally and make too many inflammatory proteins such as interleukin-6 (IL-6). This can be unicentric (one area of lymph nodes, often cured with surgery) or multicentric (many lymph node areas, often with whole-body symptoms like fever, weight loss, anaemia, and organ damage). Evidence-based guidelines show that surgery is the main treatment for unicentric disease, while biologic drugs such as siltuximab, rituximab, tocilizumab, and chemotherapy are used for multicentric disease. Austin Publishing Group+4ASHPublications+4PMC+4
Castleman disease is not a single disease. It is a group of related conditions. Some people have only one enlarged lymph node (unicentric disease). Others have many enlarged lymph nodes in different parts of the body and more severe whole-body symptoms (multicentric disease).PubMed+1
Other names for Castleman’s tumour
You may see many different names in books and research papers. They mostly mean the same basic condition:
Castleman disease / Castleman’s disease – the most common modern name.Wikipedia
Castleman tumour / Castleman’s tumour – used especially for the localized (unicentric) form that looks like a benign tumour mass.Rare Awareness Rare Education Portal
Giant lymph node hyperplasia – “giant” means very big; “hyperplasia” means too many cells growing.Wikipedia+2path.upmc.edu+2
Angiofollicular lymph node hyperplasia – “angio-” = blood vessels, “follicular” = lymphoid follicles in the node. This describes the typical blood vessel and follicle changes seen on biopsy.PubMed+1
Angiofollicular lymphoid hyperplasia / angiofollicular ganglionic hyperplasia – similar meaning; “ganglionic” refers to lymph nodes.Bangladesh Journals Online+1
Lymphoid hamartoma / hamartoma of the lymphatics – “hamartoma” means a benign over-growth of normal tissue in the wrong shape or amount.path.upmc.edu+1
Giant benign lymphoma / benign lymphoma – old terms used before doctors understood that it is not a standard cancer lymphoma.Bangladesh Journals Online+1
All of these names point to the same family of lymph node diseases.
Types of Castleman’s tumour
Doctors classify Castleman disease in several ways: by how many lymph node areas are involved, by cause, and by how the lymph node looks under the microscope.
Types by number of lymph node areas
Unicentric Castleman disease (UCD)
Only one group of lymph nodes is affected (for example, just nodes in the chest or abdomen).
Often found by accident during a scan or surgery.
Many people have no symptoms or only mild pressure symptoms from the mass.
Surgery to remove the enlarged node usually cures it.PubMed+2Wikipedia+2
Multicentric Castleman disease (MCD)
Many groups of lymph nodes are enlarged in different parts of the body.
People often have fever, weight loss, night sweats, fatigue, and organ enlargement.
It behaves like a strong inflammatory or immune disease and can be serious or even life-threatening.PubMed+2NCBI+2
Types of MCD by cause
Within multicentric disease there are three main subtypes:PubMed+2ScienceDirect+2
HHV-8-associated multicentric Castleman disease (HHV-8+ MCD)
Caused by uncontrolled infection with human herpesvirus-8 (HHV-8), also called Kaposi’s sarcoma–associated herpesvirus.Rare Awareness Rare Education Portal+2CDCN+2
Very common in people with HIV or other weak-immune states.Cleveland Clinic+1
Idiopathic multicentric Castleman disease (iMCD)
“Idiopathic” means cause unknown.
Many lymph nodes are enlarged, and there is strong inflammation, but tests for HHV-8 and other known causes are negative.PMC+2Rare Awareness Rare Education Portal+2
IL-6 and other cytokines often play a big role.Haematologica+2Wiley Online Library+2
POEMS-associated multicentric Castleman disease
POEMS is a rare syndrome: Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes.PMC+2Wiley Online Library+2
Some people with POEMS also have Castleman disease changes in their lymph nodes.
Types by how the lymph node looks (histology)
Under the microscope, the lymph node from Castleman disease can look different in each person:Wikipedia+2PubMed+2
Hyaline-vascular type
Very small or “regressed” germinal centres in the node.
Many tiny blood vessels with thick (hyaline) walls.
Lymphocytes form onion-skin layers around follicles.
Most common in unicentric disease; often few symptoms.
Plasmacytic type
More plasma cells (antibody-producing white cells) in the lymph node.
Often linked with multicentric disease.
Patients more often have fever, anemia, and high inflammatory markers.
Mixed type
Features of both hyaline-vascular and plasmacytic types.
Hypervascular (especially in iMCD)
Very rich blood supply and vessel growth.
Often linked to strong cytokine activity and inflammation.
Causes and risk factors
For many patients, the exact cause is unknown, especially in iMCD and many UCD cases. However, research has found several important triggers, risk factors, and disease mechanisms.
I will list important factors. Remember: not all are proven direct causes. Some are strong associations or mechanisms that seem to drive the disease.
Uncontrolled HHV-8 infection
HHV-8 is a herpes virus. In HHV-8-associated MCD, this virus infects cells in lymph nodes and causes them to produce large amounts of viral IL-6 and other proteins. These drive inflammation and lymph node over-growth. This is a well-proven direct cause of HHV-8+ MCD.Rare Awareness Rare Education Portal+2CDCN+2HIV infection and severe immune deficiency
People with untreated or poorly controlled HIV infection are more likely to develop HHV-8+ MCD. The weak immune system cannot control HHV-8, so the virus multiplies and triggers Castleman disease.Cleveland Clinic+2ASHPublications+2Other chronic viral infections (e.g., EBV)
Some MCD cases show co-infection with Epstein–Barr virus (EBV) and other viruses. These infections may not directly cause Castleman disease, but they can add to immune activation and may be part of the disease environment.Ovid+2ASHPublications+2Unknown trigger in idiopathic MCD (iMCD)
In iMCD, no clear infection or cancer explains the disease. Studies show that the immune system is overactive and produces many cytokines, but the starting trigger is still unknown.Rare Awareness Rare Education Portal+2CDCN+2Excess interleukin-6 (IL-6)
IL-6 is a powerful inflammatory cytokine. Many patients with multicentric Castleman disease have very high IL-6 levels, and blocking IL-6 can improve symptoms. IL-6 helps lymph nodes and plasma cells grow and makes the liver produce inflammatory proteins. It is a key driver of symptoms like fever, anemia, and high CRP.IJHMR+3PMC+3Haematologica+3Other cytokines (VEGF and others)
Molecules such as vascular endothelial growth factor (VEGF) stimulate blood vessel growth and leakage of fluid. High VEGF levels in some Castleman patients can cause fluid build-up (edema, effusions), nerve problems, and organ damage.Wikipedia+2Haematologica+2Genetic changes in lymph node stromal cells (especially UCD)
Recent studies show somatic (acquired) mutations in genes such as PDGFRB and other signalling genes in some unicentric Castleman disease cases. These mutations occur only in the lymph node tissue and may cause local over-growth, similar to a benign tumour.PMC+3Nature+3CDCN+3Abnormal signalling pathways in immune cells
Changes in signalling pathways (e.g., JAK/STAT, MAPK) in immune or stromal cells may drive cell growth and cytokine release. This can help explain why lymph nodes enlarge and the immune system stays over-active.SpringerLink+2CDCN+2Autoimmune diseases (e.g., lupus, rheumatoid arthritis)
Some patients with iMCD also have autoimmune diseases like systemic lupus erythematosus or rheumatoid arthritis. It is not clear whether these diseases cause Castleman disease or share similar immune pathways, but there is a strong link.SAGE Journals+3PMC+3SpringerLink+3Autoinflammatory diseases (e.g., Still’s disease)
Diseases with chronic systemic inflammation, such as adult-onset Still’s disease or other autoinflammatory conditions, can co-exist with iMCD or mimic it. Shared pathways of cytokine storm (IL-1, IL-6, others) may play a role.PMC+2OUP Academic+2Association with POEMS syndrome and plasma cell disorders
Some Castleman patients have POEMS syndrome or underlying plasma cell clones (abnormal antibody-producing cells). These clones may release cytokines and growth factors that contribute to Castleman disease.Frontiers+3nephropathol.com+3PMC+3Chronic immune stimulation from other infections
Chronic infections like hepatitis viruses, tuberculosis, or syphilis are sometimes seen in patients with MCD, especially in HIV-positive people. These may not directly cause the disease but can keep the immune system constantly active.ASHPublications+2ASHPublications+2Possible clonal disease of follicular dendritic cells (UCD)
In some UCD cases, the disease seems to come from a clone of follicular dendritic cells or related stromal cells in the lymph node. This again supports the idea of a benign tumour-like process in unicentric disease.EuroBloodNet+1Disordered T-cell and B-cell regulation
T-cells and B-cells control immune responses. In Castleman disease, regulation between these cells can be disturbed, leading to too many plasma cells, autoantibodies, and continuous cytokine release.PMC+2ScienceDirect+2Genetic background or susceptibility (still under study)
Some studies have found various genetic variants in Castleman patients, but no single inherited genetic cause has been confirmed. Still, some people may be more prone to abnormal immune responses based on their genes.Wikipedia+2PMC+2Hyper-cytokine states and cytokine storm conditions
Castleman disease fits within the family of “cytokine storm” disorders, where immune cells release huge amounts of cytokines, causing fever, organ damage, and lab changes. This pattern itself is an important mechanism that maintains and worsens the disease.Haematologica+2Nature+2Overlap with other lymphoproliferative disorders
Castleman disease can occur together with lymphomas, myeloma, or other blood cancers, or may be mistaken for them. Some researchers think there may be shared pathways between these conditions.Cureus+3Haematologica+3NCBI+3Chronic low-grade inflammation from unknown causes
Many Castleman patients show long periods of unexplained inflammation, even before diagnosis. This suggests that ongoing low-grade immune activation of unknown origin may be part of the cause.Environment and infections acting together with genes
It is possible that a person with a certain immune or genetic background develops Castleman disease only after exposure to some infections or environmental triggers. Research is ongoing, but this combined model is widely suspected.CDCN+2PMC+2Still-unknown factors
Even with modern research, many patients have no clear cause found. For now, doctors must accept that in many cases the disease is still “idiopathic” (cause unknown), and treatment focuses on controlling the immune storm and its complications.Nature+2Wikipedia+2
Symptoms and signs
Symptoms depend on whether the disease is unicentric or multicentric and on how active the immune system is. Some people with unicentric disease feel normal; others with multicentric disease can be very sick.
Painless swelling of lymph nodes
The most common sign is one or more enlarged, firm lymph nodes, often in the chest, neck, abdomen, or armpit. They usually do not hurt. In UCD, there may be just one mass; in MCD, many.Wikipedia+2NCBI+2Fever
Many patients with multicentric disease have recurrent or continuous fever. This comes from high levels of IL-6 and other cytokines acting on the brain’s temperature centre.Wikipedia+2Wikipedia+2Night sweats
People often wake with sweaty clothes or bed sheets, especially at night. This is another common cytokine-storm symptom.Wikipedia+1Unintended weight loss
Patients may lose weight without trying. IL-6 and other inflammatory molecules reduce appetite and change how the body uses energy and muscle.Wikipedia+2Rare Awareness Rare Education Portal+2Fatigue and weakness
Constant tiredness, weak muscles, and feeling “washed out” are very common. Causes include chronic inflammation, anemia, poor sleep from night sweats, and organ stress.Wikipedia+2SpringerLink+2Loss of appetite, nausea, or vomiting
People may feel full quickly, have poor appetite, nausea, or sometimes vomiting. Enlarged abdominal nodes or organs can press on the stomach; cytokines can also change gut function.Wikipedia+2Rare Awareness Rare Education Portal+2Abdominal fullness or pain
Enlarged lymph nodes, liver, or spleen can cause a feeling of fullness, pressure, or pain in the upper abdomen. Sometimes patients notice a lump or swelling in the belly.Mayo Clinic+2Rare Awareness Rare Education Portal+2Enlarged liver and/or spleen (hepatosplenomegaly)
Many MCD patients have a large spleen or liver on exam or scan. This happens because immune cells and abnormal blood flow collect in these organs.Wikipedia+2Rare Awareness Rare Education Portal+2Skin changes (rash, cherry hemangiomas, purple spots)
Some patients have small red or purple raised spots on the skin (cherry angiomas or violaceous papules) or other rashes. These come from abnormal blood vessels and inflammation in the skin.Rare Awareness Rare Education Portal+2SpringerLink+2Swelling of legs, abdomen, or around lungs (edema, ascites, effusions)
Excess VEGF and other factors make blood vessels leaky, so fluid leaks into tissues and body spaces. This can cause swollen legs, fluid in the belly (ascites), or fluid around the lungs (pleural effusion), leading to breathlessness.Rare Awareness Rare Education Portal+2Haematologica+2Shortness of breath or cough
Large chest lymph nodes, fluid around the lungs, or anemia can cause difficulty breathing, especially on exertion. Some patients also have lung inflammation.NCBI+2Radiology Journal+2Numbness or tingling in hands and feet (peripheral neuropathy)
In cases linked to POEMS syndrome or other neuropathies, nerves are damaged, causing tingling, burning, or weakness in the limbs.Frontiers+3Rare Awareness Rare Education Portal+3PMC+3Recurrent infections
Some patients get frequent infections (respiratory, skin, or others). This can result from abnormal immune function, low white blood cells, or damage to organs that fight infection.NCBI+2Cureus+2Anemia symptoms (pale skin, dizziness, fast heartbeat)
Many MCD patients have anemia of chronic disease. They may feel dizzy, short of breath on exertion, and look pale. Heart rate may be fast as the body tries to deliver enough oxygen.Wikipedia+2NCBI+2Joint pains and muscle aches
Some people have aches in joints and muscles, similar to autoimmune diseases. Again, this is related to high levels of inflammatory cytokines.PMC+2SpringerLink+2
Diagnostic tests
Diagnosing Castleman’s tumour is complex. There is no single blood test that proves it. Doctors combine history, physical exam, blood and urine tests, imaging, and especially lymph node biopsy. The final diagnosis always requires biopsy of an enlarged lymph node showing typical Castleman features.Wikipedia+3CDCN+3Medscape+3
Physical examination
General physical exam and vital signs
The doctor checks temperature, pulse, blood pressure, and breathing rate. Fever, fast heart rate, and low blood pressure can suggest strong inflammation or infection. They also look for weight loss, tired appearance, or breathing distress.Lymph node examination by hand (palpation)
The doctor gently feels lymph node areas (neck, armpits, groin, above clavicle) for size, number, and firmness of nodes. In Castleman disease, nodes are often firm, rubbery, and non-tender. In UCD there may be one big lump; in MCD, many enlarged nodes.NCBI+1Abdominal examination for organ enlargement
By feeling and tapping the abdomen, the doctor checks for enlarged liver or spleen and fluid in the belly. A large spleen or liver supports the idea of a systemic process like MCD.NCBI+2Rare Awareness Rare Education Portal+2
Manual / bedside clinical assessments
Functional status or performance assessment
The doctor may rate how well the patient can perform daily activities (for example, using ECOG or Karnofsky scales, even if the patient never hears the names). This helps judge how severe the illness is and how it affects daily life, which is important in MCD.Pain and tenderness assessment
The doctor asks about and gently presses areas where the patient feels pain (neck, chest, abdomen). This helps distinguish painless node swelling of Castleman disease from painful infections or abscesses and helps guide which node to biopsy.
Laboratory and pathological tests
Complete blood count (CBC)
This simple blood test measures red cells, white cells, and platelets. In Castleman disease there may be anemia, high or low platelet counts, and various white cell changes. The exact pattern can help separate subtypes and rule out leukemias and lymphomas.NCBI+2Wikipedia+2Inflammatory markers: ESR and C-reactive protein (CRP)
These tests measure inflammation in the body. Most MCD patients have very high ESR or CRP levels because of IL-6 and other cytokines. These tests also help track how well treatment is working.PMC+2NCBI+2Liver function tests (LFTs)
This panel (e.g., ALT, AST, alkaline phosphatase, bilirubin, albumin) checks how the liver is working. Castleman disease can cause low albumin, raised enzymes, or cholestasis if the liver is involved or if inflammation is severe.NCBI+2Wikipedia+2Kidney function tests (creatinine, urea)
These blood tests show how well the kidneys filter waste. Some patients with MCD or POEMS-associated disease have kidney damage or protein loss in urine. Abnormal results may change treatment and prognosis.nephropathol.com+2Cureus+2Serum protein electrophoresis
This test separates blood proteins and looks for abnormal bands (monoclonal proteins) and overall shape of the protein curve. In Castleman disease there may be high globulin levels and sometimes a monoclonal band, especially in POEMS or plasma-cell–rich variants.Wikipedia+2nephropathol.com+2Immunoglobulin (antibody) levels
Levels of IgG, IgA, IgM may be high in many MCD patients (hypergammaglobulinemia). This shows that B-cells and plasma cells are overactive. It also helps separate Castleman disease from other causes of inflammation.Wikipedia+2PMC+2Viral tests for HIV and hepatitis
Testing for HIV, hepatitis B, and hepatitis C is important, because HIV strongly increases the risk of HHV-8+ MCD, and hepatitis viruses may be associated or affect treatment choices.Cleveland Clinic+2ASHPublications+2HHV-8 testing (PCR or immunostaining)
To decide whether the patient has HHV-8-associated MCD or iMCD, doctors test for HHV-8 in blood or tissue using PCR or special stains. A positive result with the right clinical picture supports HHV-8+ MCD; a negative test helps point towards iMCD.Rare Awareness Rare Education Portal+2CDCN+2Cytokine levels: IL-6 (± VEGF and others)
Some centres measure IL-6 and VEGF levels. High levels support the idea of a cytokine-driven Castleman disease and may correlate with severity. However, normal levels do not completely rule out the disease.ResearchGate+3Haematologica+3Wiley Online Library+3Lymph node excisional biopsy with histopathology
This is the key test. A surgeon removes all or part of an enlarged lymph node. A pathologist examines it under the microscope with special stains. They look for Castleman features such as regressed germinal centres, onion-skin mantle zones, and increased blood vessels or plasma cells. Without this biopsy, the diagnosis cannot be confirmed.ASHPublications+3CDCN+3path.upmc.edu+3Bone marrow biopsy
A needle sample from the pelvic bone shows if the bone marrow is affected. Changes may include increased plasma cells, abnormal megakaryocytes, fibrosis, or features of associated disorders like POEMS or myelodysplasia. This helps staging and planning treatment.NCBI+2nephropathol.com+2
Electrodiagnostic tests
Nerve conduction studies and electromyography (EMG)
When patients have numbness, tingling, or weakness in arms or legs, nerve conduction tests and EMG show how well the nerves and muscles work. They can confirm polyneuropathy, which is important in POEMS-associated Castleman disease and helps guide therapy.PMC+2ejcrim.com+2Electrocardiogram (ECG)
An ECG records the heart’s electrical activity. It is not specific for Castleman disease, but it is useful to check for heart strain, rhythm problems, or effects of anemia, fluid overload, or some treatments. Doctors often include it in the general workup of a sick MCD patient.
Imaging tests
Computed tomography (CT) scan of neck, chest, abdomen, and pelvis
CT is one of the main imaging tests. It shows the size, number, and location of enlarged lymph nodes and whether the disease is unicentric (one region) or multicentric (many regions). CT also checks for organ enlargement and guides the choice of node for biopsy.Clinical Radiology Online+3Mayo Clinic+3Radiology Journal+318F-FDG PET/CT scan
PET/CT combines metabolic and structural imaging. FDG-PET shows how “active” a lymph node or lesion is by measuring glucose uptake. In Castleman disease, PET/CT helps:Find all involved nodes,
Select the best node to biopsy,
Monitor response to treatment.SpringerOpen+2ejcrim.com+2
Ultrasound of abdomen
Ultrasound uses sound waves to look at abdominal organs and nodes. It can detect enlarged liver, spleen, and abdominal lymph nodes and is helpful as a first, non-invasive test, especially in children or pregnant people.Radiology Journal+1Chest X-ray
A simple chest X-ray may show a large mediastinal mass (big lymph node in the centre of the chest) or fluid around the lungs. It is often an early clue that leads to further CT or PET/CT imaging.Radiology Journal+2webpathology.com+2
Non-pharmacological treatments
1. Curative surgical removal of the affected lymph node
For unicentric Castleman disease, complete surgical removal of the enlarged lymph node is usually the first and most effective treatment. Studies and guidelines show that removing the single diseased node often cures the condition, with long-term remission and symptom relief. Wikipedia+3ScienceDirect+3Austin Publishing Group+3 The purpose is to remove the abnormal lymph tissue that is over-producing IL-6 and other inflammatory proteins. The mechanism is purely mechanical: once the node is removed, the main source of excess cytokines disappears, which allows fever, fatigue, anaemia, and high inflammatory markers to settle. Even though this is not a drug, it is the cornerstone non-pharmacological therapy for most localized Castleman’s tumours.
2. Radiation therapy for unresectable or high-risk unicentric disease
When the Castleman mass cannot be safely removed by surgery—because of location, size, or patient risk—radiation therapy is a key option. Case series show that doses around 27–45 Gy can achieve good response or remission in many patients with unresectable unicentric Castleman disease. Lippincott Journals+4PMC+4ResearchGate+4 Radiation’s purpose is to shrink the lymph node mass, reduce pain or compression, and lower cytokine production. Mechanistically, focused radiation damages the abnormal lymphoid cells’ DNA so they cannot divide, gradually shrinking the tumour. This can relieve pressure on nearby organs and improve symptoms like cough, chest pain, or abdominal discomfort.
3. Watchful waiting in stable, mild cases
In a few patients with very mild, stable Castleman disease, especially after successful treatment, doctors may choose careful observation instead of immediate further therapy. The purpose is to avoid unnecessary treatment risks while still catching relapse early. Guidelines mention that asymptomatic or minimally symptomatic unicentric disease after resection and some mild iMCD cases may be followed with regular exams, blood tests, and scans. Medscape+2ASHPublications+2 The mechanism is not biological but strategic: frequent monitoring allows timely treatment if the lymph nodes grow again or if systemic symptoms appear, while respecting the fact that many patients can remain well for years without continuous therapy.
4. Multidisciplinary supportive care team
Because Castleman disease can affect blood counts, liver, kidneys, lungs, and general wellbeing, a multidisciplinary team (haematologist, oncologist, infectious disease specialist, rheumatologist, dietitian, physiotherapist, psychologist) is highly recommended. Expert sources emphasise that optimal management often requires coordinated care to monitor complications such as infections, thrombosis, organ failure, and overlapping autoimmune features. Wiley Online Library+4ASHPublications+4PMC+4 The purpose is to cover all organs and symptoms. The mechanism is organisational: better communication between specialists leads to better decisions about when to adjust biologic therapy, when to treat infections aggressively, and how to support nutrition and mental health.
5. Structured infection-prevention and vaccination programme
Patients with multicentric Castleman disease often receive immunosuppressive therapies and have low blood counts, making infections more dangerous. Guidelines for similar haematologic conditions recommend up-to-date vaccines (influenza, pneumococcal, COVID-19, hepatitis B when appropriate) and prompt treatment of bacterial or viral infections. Haematologica+4Rare Awareness Rare Education Portal+4CDCN+4 The purpose is to reduce pneumonia, sepsis, and opportunistic infections. Mechanistically, vaccines train the immune system to recognise pathogens before exposure, while early antibiotics or antivirals limit spread when infection occurs. Preventing infections also helps avoid flares of Castleman-related inflammation.
6. Nutritional optimisation and dietetic therapy
Many Castleman patients suffer from weight loss, low albumin, and muscle wasting due to chronic inflammation and poor appetite. Wikipedia+4ASHPublications+4PMC+4 A dietitian can design high-protein, high-calorie, anti-inflammatory meal plans tailored to the patient’s culture and preferences. The purpose is to rebuild strength, support immune function, and prepare the body for chemotherapy or biologic infusions. Mechanistically, adequate protein and micronutrients (iron, folate, vitamin D, zinc) support red blood cell production, wound healing, and immune cell function, while balanced carbohydrates and healthy fats provide steady energy and may reduce fatigue.
7. Physical therapy and graded exercise
Chronic fatigue, anaemia, and muscle loss are common in multicentric Castleman disease. Journal of Pediatrics+4ASHPublications+4Medscape+4 Physiotherapy and gentle exercise programmes can safely rebuild stamina and mobility. The purpose is to reduce deconditioning, joint stiffness, and risk of blood clots from prolonged bed rest. Mechanistically, graded aerobic and resistance exercise improves muscle mass, mitochondrial function, and circulation, which can lessen fatigue and improve mood. Exercise also supports bone health, which is important when long-term steroids or inflammation increase osteoporosis risk.
8. Psychological counselling and mental health support
Castleman’s tumour is rare and often frightening, and many patients experience anxiety or depression. Qualitative research in similar chronic haematologic diseases shows that psychological support, cognitive-behavioural therapy, and peer support groups improve quality of life and treatment adherence. Haematologica+4ASHPublications+4PMC+4 The purpose is to help patients cope with uncertainty, manage fear of relapse, and adjust to long-term follow-up. Mechanistically, improved mental health reduces stress-hormone levels and improves sleep and self-care, which indirectly support immune function and recovery.
9. Sleep hygiene and fatigue-management strategies
Systemic inflammation and steroids can disturb sleep in Castleman patients. Good sleep hygiene (regular bedtime, dark quiet room, limiting screens and caffeine, wind-down routine) is recommended in chronic inflammatory disorders to manage fatigue. Wikipedia+4ASHPublications+4PMC+4 The purpose is to improve energy, mood, and concentration. Mechanistically, deep sleep helps regulate immune responses, hormone release, and tissue repair; poor sleep can worsen pain, depression, and inflammatory markers, so non-drug sleep interventions are an important supportive therapy.
10. Smoking cessation and alcohol moderation
Smoking and heavy alcohol use worsen cardiovascular risk, lung function, liver damage, and infection risk—problems that Castleman patients already face because of inflammation and some treatments. Wikipedia+4ASHPublications+4Medscape+4 The purpose of stopping smoking and reducing alcohol is to lower complications like pneumonia, blood clots, heart disease, and cirrhosis. Mechanistically, stopping toxins reduces oxidative stress and chronic inflammation, allowing blood vessels, lungs, and liver to function better and tolerate chemotherapy or biologic drugs.
11. Stress-reduction techniques (mindfulness, relaxation, yoga, breathing)
Chronic stress activates the sympathetic nervous system and raises inflammatory markers, which can worsen symptoms in immune-mediated diseases. Small studies in cancer and autoimmune disorders show that mindfulness, gentle yoga, and breathing techniques can lower anxiety and improve perceived quality of life. Wiley Online Library+4ASHPublications+4PMC+4 The purpose is not to cure Castleman disease but to help the nervous system “down-shift.” Mechanistically, deep breathing and relaxation modulate autonomic tone, reduce cortisol levels, and may indirectly dampen inflammatory signalling.
12. Pain-management strategies (non-drug methods)
Some patients feel pain from large lymph nodes, surgery, or neuropathy from chemotherapy. Non-drug methods such as heat pads, cold packs, gentle massage (if safe), posture correction, and distraction techniques can reduce pain and limit the need for strong painkillers. Haematologica+4PMC+4Austin Publishing Group+4 The purpose is symptom relief and better function. Mechanistically, heat relaxes muscles and improves blood flow; cold reduces swelling; massage stimulates touch fibres that “compete” with pain signals in the spinal cord; and distraction changes how the brain interprets pain.
13. Thrombosis prevention (movement, stockings, hydration)
Castleman disease and some treatments increase the risk of blood clots. Expert reviews of iMCD stress monitoring for thrombosis and using standard preventive measures, especially when patients are hospitalised or very inactive. JCI+4ASHPublications+4PMC+4 The purpose of non-drug prevention—regular walking, leg exercises, compression stockings, and good hydration—is to keep blood flowing smoothly. Mechanistically, muscle movement and compression enhance venous return, reducing pooling in the legs where clots commonly form.
14. Infection-control practices at home
Because many patients are immunosuppressed, simple infection-control steps at home are very important: hand washing, avoiding close contact with sick people, careful food hygiene, and prompt attention to fevers. This approach follows general infection-prevention guidance used in other immunocompromised conditions. Haematologica+4Rare Awareness Rare Education Portal+4CDCN+4 The purpose is to reduce the chance of severe respiratory or gut infections, which can trigger Castleman flares and hospitalisation. Mechanistically, fewer pathogen exposures mean fewer infections, less systemic inflammation, and safer continuation of biologic or chemotherapy regimens.
15. Rehabilitation after surgery or intensive treatment
After major lymph-node surgery, stem-cell transplant, or intensive chemotherapy, many patients benefit from structured rehabilitation. This may include physiotherapy, occupational therapy, and nutritional and psychological support. Haematologica+4ScienceDirect+4Lippincott Journals+4 The purpose is to speed recovery, restore independence in daily activities, and reduce long-term disability. Mechanistically, rehab uses repeated, graded activity to retrain muscles and nerves, while teaching energy-saving techniques to cope with lingering fatigue.
16. Social work and financial support services
Castleman disease often requires long treatment and frequent hospital visits. Social workers can help patients access disability benefits, transport assistance, work accommodations, and home help services. Health-policy research shows that practical support reduces treatment interruptions and improves outcomes in chronic cancer-like illnesses. Haematologica+4ASHPublications+4PMC+4 The purpose is to reduce the financial and logistical burden, which often worsens stress and mental health. Mechanistically, reducing these pressures allows patients to attend appointments consistently and follow complex treatment plans.
17. Palliative care for symptom control in advanced cases
In some advanced or treatment-resistant multicentric Castleman cases, palliative care is essential. Palliative specialists focus on controlling pain, breathlessness, fatigue, and emotional distress while respecting the patient’s goals. This approach is recommended in guidelines for other life-threatening haematologic disorders and is increasingly discussed in Castleman disease reviews. Wiley Online Library+4ASHPublications+4PMC+4 The purpose is to improve comfort, not to give up on treatment. Mechanistically, symptom control allows better sleep, eating, and family interaction, which can improve overall quality of life even when cure is not possible.
18. Education and self-management training
Because Castleman disease is rare, many patients have never heard of it before diagnosis. Reliable organizations such as the Castleman Disease Collaborative Network (CDCN) and academic centres provide education on symptoms, treatments, and warning signs. Wikipedia+4CDCN+4ASHPublications+4 The purpose is to empower patients to notice early relapse, side effects, or infections. Mechanistically, informed patients make safer choices—such as seeking help quickly for fevers—and communicate better with their doctors, which improves outcomes.
19. Fertility preservation counselling
Some Castleman treatments (for example, alkylating chemotherapy) can harm fertility. In line with oncology good practice, patients of childbearing age should be counselled about sperm banking or egg/embryo freezing before intensive therapy. Wiley Online Library+4cancertherapyadvisor.com+4Dove Medical Press+4 The purpose is to give patients future reproductive options. Mechanistically, freezing gametes before treatment avoids the risk of chemotherapy-induced gonadal damage. This non-pharmacological step can have huge long-term emotional benefits.
20. Participation in clinical trials
Because Castleman disease is rare, clinical trials are crucial to improving care. There are ongoing and completed trials of agents such as sirolimus and other targeted therapies for idiopathic multicentric disease. SpringerLink+4ClinicalTrials.gov+4CDCN+4 The purpose of joining a trial is to access promising new treatments and contribute to scientific knowledge. Mechanistically, many of these drugs act on specific pathways like mTOR or BTK that are abnormally activated in Castleman disease, aiming to control inflammation and lymph-node growth more precisely than older chemotherapies.
Drug treatments
Important: Doses below are typical examples from studies/labels, not personal prescriptions. Your own dose and schedule must be set by your specialist.
1. Siltuximab (SYLVANT) – IL-6 antagonist
Class: Monoclonal antibody against interleukin-6 (IL-6).
Evidence & use: Siltuximab is the only FDA-approved drug specifically for idiopathic multicentric Castleman disease (iMCD) in HIV-negative, HHV-8-negative adults. Louisiana Department of Health+4FDA Access Data+4FDA Access Data+4
Typical dose/time: 11 mg/kg as an intravenous infusion over 1 hour every 3 weeks until treatment failure.
Purpose: To block IL-6, a key cytokine driving fever, anaemia, enlarged nodes, and high CRP in iMCD. Wikipedia+4ASHPublications+4PMC+4
Mechanism: By binding IL-6, siltuximab prevents it from activating its receptor, reducing inflammatory signalling, lymph-node overgrowth, and systemic symptoms.
Side effects: Infusion reactions, infections, low white counts, abnormal liver tests, and gastrointestinal symptoms are reported; patients require regular monitoring and infection precautions. Maryland.gov Enterprise Agency Template+4FDA Access Data+4FDA Access Data+4
2. Rituximab – anti-CD20 B-cell depleting antibody
Class: Anti-CD20 monoclonal antibody.
Evidence & use: Rituximab is first-line therapy for HHV-8-positive multicentric Castleman disease, especially in people living with HIV, and is also used in some iMCD and overlap cases. Wiley Online Library+4CDCN+4Mayo Clinic+4
Typical dose/time: Common regimens use 375 mg/m² IV weekly for 4 weeks, or combined with chemotherapy (e.g., R-CHOP). Ovid+4cancertherapyadvisor.com+4CDCN+4
Purpose: To remove CD20-positive B cells that harbour HHV-8 and produce excess IL-6 and related cytokines.
Mechanism: Rituximab binds CD20 on B cells, leading to immune-mediated cell killing and depletion.
Side effects: Infusion reactions, hepatitis B reactivation, infections, low blood counts, and rare severe skin or heart reactions; careful screening is essential. Wikipedia+4Wiley Online Library+4Mayo Clinic+4
3. Tocilizumab – IL-6 receptor blocker (off-label)
Class: Humanized monoclonal antibody against the IL-6 receptor.
Evidence & use: Case series show that tocilizumab can improve symptoms and lab tests in multicentric Castleman disease, especially when siltuximab is unavailable or ineffective. Frontiers+4PubMed+4Haematologica+4
Typical dose/time: 8 mg/kg IV every 2 weeks has been reported, sometimes adjusted by response. ASHPublications+4PubMed+4Haematologica+4
Purpose: To block IL-6 signalling at the receptor level and control inflammation.
Mechanism: Tocilizumab binds IL-6 receptor (membrane and soluble forms), preventing IL-6 from activating JAK/STAT pathways that drive anaemia, fever, and lymph-node growth.
Side effects: Infection risk, elevated liver enzymes, high lipids, gastrointestinal perforation in high-risk patients, and infusion reactions; regular monitoring is required.
4. Prednisone and other systemic corticosteroids
Class: Glucocorticoid anti-inflammatory drugs.
Evidence & use: Corticosteroids alone rarely give durable remission but are widely used to rapidly control symptoms or as part of combination regimens in iMCD and HHV-8+ MCD. CDCN+4Medscape+4ASHPublications+4
Typical dose/time: Regimens vary; examples include 0.5–1 mg/kg/day orally, or high-dose pulses IV, then tapering.
Purpose: Quick reduction of fever, inflammation, and organ dysfunction while definitive therapy (like siltuximab or rituximab) is started.
Mechanism: Steroids suppress many inflammatory genes and immune cells, reducing cytokine release and vascular leak.
Side effects: Weight gain, high blood sugar, mood changes, osteoporosis, infection risk, and adrenal suppression; they must be tapered, not stopped suddenly.
5. Cyclophosphamide
Class: Alkylating chemotherapy agent.
Evidence & use: Cyclophosphamide is a key component of CHOP-based regimens used for aggressive or refractory multicentric Castleman disease, often combined with rituximab. WMJ+4cancertherapyadvisor.com+4Dove Medical Press+4
Typical dose/time: In CHOP, cyclophosphamide is infused IV every 21 days at weight-based doses, usually for 4–6 cycles.
Purpose: To target rapidly dividing lymphoid cells driving Castleman disease.
Mechanism: It cross-links DNA, causing cell death, especially in proliferating B and plasma cells in lymph nodes.
Side effects: Nausea, hair loss, low blood counts, infertility risk, bladder irritation, and secondary malignancy risk; protective measures like hydration and monitoring are used.
6. Doxorubicin (Adriamycin)
Class: Anthracycline chemotherapy.
Evidence & use: Doxorubicin is another CHOP component used in certain aggressive or overlapping Castleman presentations, often in R-CHOP protocols. WMJ+4cancertherapyadvisor.com+4Dove Medical Press+4
Typical dose/time: Given IV on day 1 of each 21-day cycle.
Purpose: To further kill proliferating lymphoid cells and improve control of the disease, especially when lymphoma-like features are present.
Mechanism: Intercalates into DNA and inhibits topoisomerase II, producing free radicals that damage cancerous cells.
Side effects: Heart toxicity, hair loss, low blood counts, mouth sores, and nausea; cumulative dose is carefully limited and heart function is monitored.
7. Vincristine
Class: Vinca alkaloid chemotherapy.
Evidence & use: Vincristine is part of CHOP and related regimens used in some Castleman patients with severe or refractory disease. WMJ+4cancertherapyadvisor.com+4Dove Medical Press+4
Typical dose/time: Small IV doses on day 1 of each chemotherapy cycle.
Purpose: To enhance anti-tumour effects within combination chemotherapy.
Mechanism: It binds tubulin and blocks microtubule formation, stopping cell division in metaphase and triggering cell death.
Side effects: Peripheral neuropathy (tingling, numbness), constipation, low blood counts, and hair loss; doses are adjusted if nerve symptoms appear.
8. Etoposide
Class: Topoisomerase II inhibitor chemotherapy.
Evidence & use: Etoposide is sometimes added to CHOP-like regimens (e.g., CHOEP or R-CHOEP) for more aggressive disease or relapsed iMCD. Ovid+4Dove Medical Press+4CDCN+4
Typical dose/time: IV or oral on several days of each cycle, using weight-based dosing.
Purpose: To intensify cytotoxic activity against proliferating lymphoid cells.
Mechanism: Etoposide interferes with topoisomerase II, causing DNA breaks during replication and leading to apoptosis.
Side effects: Low blood counts, hair loss, nausea, and risk of secondary leukaemia with high cumulative doses.
9. Thalidomide
Class: Immunomodulatory and anti-angiogenic agent.
Evidence & use: Case reports and small series show dramatic responses to thalidomide in some multicentric Castleman patients, especially steroid-resistant cases. Nature+3PubMed+3Wiley Online Library+3
Typical dose/time: Oral daily dosing (e.g., 50–200 mg) adjusted to tolerance in studies.
Purpose: To disrupt cytokine and blood-vessel growth signals that feed the disease.
Mechanism: Thalidomide reduces IL-6 and TNF-α production and inhibits angiogenesis, helping shrink lymph nodes and relieve systemic symptoms.
Side effects: Severe birth defects (absolute pregnancy contraindication), neuropathy, constipation, drowsiness, blood clots; strict pregnancy-prevention programmes are mandatory.
10. Lenalidomide
Class: Second-generation immunomodulatory drug (IMiD).
Evidence & use: Small retrospective series and case reports suggest lenalidomide-containing regimens can induce remission in relapsed/refractory multicentric Castleman disease. Directory of Open Access Journals+3e-Century Publishing+3Lippincott Journals+3
Typical dose/time: Oral 10–25 mg daily on days 1–21 of a 28-day cycle in reports, often combined with steroids or tocilizumab.
Purpose: To modulate immune and angiogenic pathways when IL-6-directed strategies or chemotherapy are insufficient.
Mechanism: Lenalidomide affects cereblon-dependent signalling, alters cytokine production, boosts certain T-cell responses, and inhibits abnormal blood-vessel formation.
Side effects: Low blood counts, blood clots, rash, fatigue; pregnancy prevention is essential.
11. Bortezomib
Class: Proteasome inhibitor.
Evidence & use: Case reports describe successful treatment of multicentric Castleman disease associated with multiple myeloma using bortezomib-based regimens. Haematologica+3Nature+3e-Century Publishing+3
Typical dose/time: Given IV or subcutaneously on specific days of 21- or 28-day cycles, often with steroids.
Purpose: To target plasma cells and abnormal antibody production, especially when Castleman disease overlaps with plasma-cell disorders.
Mechanism: Bortezomib blocks proteasomes, leading to accumulation of misfolded proteins and apoptosis in plasma cells.
Side effects: Peripheral neuropathy, low platelets, fatigue, diarrhoea or constipation, and infection risk.
12. Interferon-alpha
Class: Cytokine-based immunotherapy.
Evidence & use: Older series report remission of HIV- and HHV-8-associated multicentric Castleman disease with interferon-alpha, sometimes as first-line therapy before rituximab became standard. Haematologica+4ScienceDirect+4Nature+4
Typical dose/time: Subcutaneous injections several times per week in historical protocols.
Purpose: To exert antiviral and anti-proliferative effects, particularly against HHV-8-driven disease.
Mechanism: Interferon-alpha enhances antiviral responses, modulates immune cells, and can slow proliferation of abnormal lymphoid cells.
Side effects: Flu-like symptoms, depression, thyroid dysfunction, and blood-count suppression.
13. Sirolimus (mTOR inhibitor) – for refractory iMCD
Class: mTOR pathway inhibitor.
Evidence & use: Recent mechanistic and clinical studies show increased mTOR activation in iMCD; small series and retrospective studies report sirolimus as effective in anti-IL-6-refractory iMCD. CDCN+4ASHPublications+4JCI+4
Typical dose/time: Oral daily dosing adjusted to blood trough levels, often used in clinical-trial or specialist settings.
Purpose: To control disease when siltuximab or tocilizumab fails or cannot be used.
Mechanism: Inhibits mTOR, a central growth and metabolism regulator, reducing lymph-node proliferation and cytokine production.
Side effects: Mouth ulcers, high lipids, kidney dysfunction, wound-healing delay, and infection risk; drug levels and labs must be monitored.
14. Antiviral drugs (e.g., ganciclovir, valganciclovir)
Class: Antiviral agents against herpesviruses.
Evidence & use: HHV-8-positive MCD sometimes improves with antivirals targeting HHV-8, often alongside rituximab and chemotherapy. MDPI+4Rare Awareness Rare Education Portal+4CDCN+4
Typical dose/time: IV or oral regimens similar to those used for CMV, tailored by infectious-disease specialists.
Purpose: To reduce viral replication that contributes to IL-6 overproduction and Castleman flares.
Mechanism: Ganciclovir-like drugs inhibit viral DNA polymerase, reducing HHV-8 replication in infected B cells.
Side effects: Bone-marrow suppression, kidney toxicity, and gastrointestinal symptoms; careful monitoring is required.
15. Combination antiretroviral therapy (ART) for HIV
Class: Mixed antiviral drug classes.
Evidence & use: In HIV-associated MCD, effective ART is crucial. Case reports show that starting or optimising ART can help control HHV-8 replication and improve Castleman disease outcomes, especially when combined with rituximab or chemotherapy. PMC+4PMC+4ScienceDirect+4
Typical dose/time: Daily oral combinations (e.g., integrase inhibitor plus two NRTIs), per HIV guidelines.
Purpose: To restore immune function and reduce HHV-8 activity.
Mechanism: By suppressing HIV, ART improves CD4 counts and immune control, which indirectly limits HHV-8-driven Castleman activity.
Side effects: Depend on regimen but may include nausea, liver toxicity, lipid changes, and drug interactions.
16. Azathioprine (selective use)
Class: Thiopurine immunosuppressant.
Evidence & use: Azathioprine has been tried in some Castleman cases, often as maintenance after more intensive therapy, with mixed results. ScienceDirect+4BioMed Central+4AACR Journals+4
Typical dose/time: Oral daily dosing based on weight, with regular blood monitoring.
Purpose: To maintain disease control while reducing steroid exposure.
Mechanism: Azathioprine is converted to 6-mercaptopurine, which interferes with purine synthesis and reduces lymphocyte proliferation.
Side effects: Bone-marrow suppression, liver toxicity, infection risk, nausea, and rare pancreatitis.
17. Orelabrutinib and other BTK inhibitors (investigational)
Class: Bruton’s tyrosine kinase (BTK) inhibitors.
Evidence & use: Early retrospective studies suggest BTK inhibitors such as orelabrutinib may help relapsed/refractory iMCD by targeting B-cell receptor signalling. PMC+4ResearchGate+4ASHPublications+4
Typical dose/time: Oral daily tablets in clinical trials.
Purpose: To offer a non-cytotoxic option for difficult-to-treat patients.
Mechanism: BTK inhibition interferes with B-cell survival and activation, reducing cytokine and antibody production.
Side effects: Generally include infections, bleeding tendency, diarrhoea, and liver enzyme elevations.
18. Intravenous immunoglobulin (IVIG)
Class: Pooled human immunoglobulin.
Evidence & use: Not specific to Castleman disease, but IVIG is sometimes used when patients have recurrent infections or antibody deficiencies after intensive immunosuppression. Wikipedia+4ASHPublications+4PMC+4
Typical dose/time: High-dose IV infusions every 3–4 weeks, under specialist supervision.
Purpose: To provide passive immunity and reduce serious infections that can worsen Castleman disease.
Mechanism: IVIG supplies a broad mix of antibodies that help neutralise bacteria and viruses and modulate immune responses.
Side effects: Headache, infusion reactions, kidney strain, and rare thrombosis.
19. Prophylactic antibiotics or antifungals (selected patients)
Class: Anti-infective drugs.
Evidence & use: In heavily immunosuppressed patients (e.g., after chemotherapy or high-dose steroids), prophylactic antibiotics or antifungals may be used according to standard haematology protocols. Haematologica+4Rare Awareness Rare Education Portal+4CDCN+4
Typical dose/time: Daily oral or intermittent dosing (e.g., trimethoprim-sulfamethoxazole to prevent Pneumocystis pneumonia).
Purpose: To prevent opportunistic infections that could trigger severe flares or organ failure.
Mechanism: These drugs block specific microbial pathways, reducing the chance of infection taking hold in a weakened host.
Side effects: Allergic reactions, gastrointestinal upset, and drug interactions.
20. Supportive drugs (e.g., proton-pump inhibitors, bone-protective agents)
Class: Symptom- and toxicity-management medications.
Evidence & use: Guidelines for cancer and chronic inflammatory conditions recommend PPIs to protect the stomach on steroids/NSAIDs and bisphosphonates or vitamin D for steroid-induced bone loss. Wikipedia+3Medscape+3PMC+3
Typical dose/time: Daily oral tablets as prescribed.
Purpose: To reduce complications of necessary Castleman treatments rather than the disease itself.
Mechanism: PPIs reduce stomach acid; bisphosphonates slow bone breakdown; vitamin D improves calcium use.
Side effects: PPIs may raise infection risk; bisphosphonates can cause bone or jaw pain; vitamin D excess can disturb calcium levels.
Dietary molecular supplements
For Castleman disease there are no supplements proven to cure or directly control the disease, but some nutrients have general immune or anti-inflammatory benefits based on broader literature. Always discuss supplements with your doctor, especially with chemotherapy or biologics.
Vitamin D – Low vitamin D is common in chronic inflammatory and haematologic conditions, and correction supports bone and immune health. Typical doses range from 600–2000 IU/day, adjusted by blood level. Vitamin D affects gene expression in immune cells, helping balance pro- and anti-inflammatory responses. Wikipedia+4Medscape+4PMC+4
Omega-3 fatty acids (EPA/DHA) – Found in fish oil, these fats have been shown to lower inflammatory markers and may modestly improve symptoms in chronic inflammatory diseases. Doses of 1–3 g/day of combined EPA/DHA are common. Omega-3s compete with omega-6 fatty acids in cell membranes, leading to production of less-inflammatory eicosanoids and specialised pro-resolving mediators. ASHPublications+4Medscape+4PMC+4
Curcumin (from turmeric) – Curcumin has broad anti-inflammatory and antioxidant effects in preclinical and small human studies. It may inhibit NF-κB and reduce cytokines such as IL-6 and TNF-α. Typical supplement doses are 500–1000 mg/day with absorption enhancers like piperine. Haematologica+4PMC+4ASHPublications+4
Green tea catechins (EGCG) – Epigallocatechin gallate has antioxidant and immune-modulating properties in experimental models, including effects on B-cell signalling and angiogenesis. Common doses are 200–400 mg/day of standardized extract, avoiding very high doses that may stress the liver. Haematologica+4ASHPublications+4JCI+4
Probiotics (multi-strain) – Gut microbiome balance is important for immune regulation. Probiotic mixtures containing Lactobacillus and Bifidobacterium strains can improve some markers of inflammation and diarrhoea in other conditions. Mechanistically, they interact with gut immune cells and strengthen the intestinal barrier. Typical use is once-daily capsules or fermented foods. JCI+4PMC+4Wikipedia+4
Zinc – Zinc deficiency impairs immune function and wound healing. Supplementation (usually 10–25 mg/day) can correct deficiency but high doses should be avoided long-term. Zinc is required for many immune enzymes and transcription factors. JCI+4PMC+4Wikipedia+4
Selenium – Selenium is an essential trace element with antioxidant roles via selenoproteins like glutathione peroxidases. Adequate selenium supports immune responses and may improve tolerance of oxidative stress during treatment. Typical doses are 50–200 mcg/day. JCI+4PMC+4Wikipedia+4
N-acetylcysteine (NAC) – NAC replenishes glutathione, a key intracellular antioxidant, and has mucolytic and potential anti-inflammatory properties. Doses in other chronic diseases often range 600–1800 mg/day. NAC may help protect organs from oxidative damage associated with chronic inflammation and some chemotherapies. Wikipedia+4PMC+4Haematologica+4
Coenzyme Q10 (CoQ10) – CoQ10 supports mitochondrial energy production and has antioxidant effects. Small trials in other conditions show reduced fatigue and improved exercise capacity. Typical doses are 100–300 mg/day. This may help Castleman patients struggling with severe fatigue, though direct evidence in Castleman disease is lacking. JCI+4PMC+4Haematologica+4
Medical oral nutrition supplements – High-protein shakes containing balanced amino acids, vitamins, and minerals can be crucial for patients with poor appetite. They provide 200–400 kcal and 10–20 g protein per serving, supporting muscle maintenance and immune function during treatment. Wikipedia+4Medscape+4PMC+4
Immunity-booster / regenerative / stem-cell–related drugs
Again, these are specialist therapies and not general “wellness boosters.”
Filgrastim (G-CSF) – A granulocyte colony-stimulating factor used when Castleman treatments cause severe neutropenia. It is injected subcutaneously daily or a few times per week. It stimulates the bone marrow to produce neutrophils, reducing infection risk and allowing chemotherapy or biologic therapy to continue. Wikipedia+3Medscape+3PMC+3
Plerixafor with G-CSF (for stem-cell mobilisation) – In patients who need autologous stem-cell transplantation, plerixafor plus G-CSF helps release stem cells from bone marrow into blood for collection. It blocks CXCR4/SDF-1 interaction, allowing more CD34+ cells to circulate. ASHPublications+4Medscape+4JCI+4
Eltrombopag or romiplostim (thrombopoietin receptor agonists) – When Castleman disease and its treatments cause severe thrombocytopenia, these drugs stimulate platelet production. Taken orally (eltrombopag) or by injection (romiplostim), they activate TPO receptors in the marrow, encouraging megakaryocyte growth. Wikipedia+4Medscape+4PMC+4
Intravenous immunoglobulin (IVIG) – Already described above, IVIG can be considered a regenerative “immune replacement” when antibody levels are low or infection risk is high. It provides functional antibodies and modulates auto-antibodies, improving immune defence. Wikipedia+4ASHPublications+4PMC+4
Hematopoietic stem-cell transplantation (HSCT) – drug-supported procedure – HSCT is rarely used but may be considered in very aggressive, refractory Castleman cases or when overlapping haematologic malignancy is present. High-dose chemotherapy is followed by infusion of stem cells (autologous or allogeneic), supported by G-CSF and prophylactic drugs. This “resets” the immune and blood-forming system. Haematologica+4BioMed Central+4Wiley Online Library+4
Sirolimus (as a regenerative/immune-modulating precision therapy) – Already discussed in drug section, sirolimus can be seen as a “regenerative-friendly” agent because, by normalising mTOR signalling, it may restore healthier immune and growth patterns in refractory iMCD. ResearchGate+4ASHPublications+4JCI+4
Surgeries (procedures)
1. Complete lymph-node excision (curative surgery for unicentric disease)
This is the classic operation for unicentric Castleman’s tumour. The surgeon makes an incision directly over the affected area (neck, chest, abdomen, or retroperitoneum) and removes the enlarged lymph node and any involved surrounding tissue. Wikipedia+4ScienceDirect+4Austin Publishing Group+4 The goal is complete removal, which often cures the disease and prevents recurrence. It is done because the single node is the main source of excess IL-6 and inflammation; once removed, symptoms such as fever and anaemia often resolve.
2. Laparoscopic lymph-node resection
When Castleman’s tumour is in the abdomen or pelvis, a laparoscopic (keyhole) approach may be used. Small incisions are made, and a camera and instruments are inserted to carefully dissect and remove the node. Lippincott Journals+4ScienceDirect+4Cureus+4 The procedure is chosen to reduce pain, hospital stay, and scarring while achieving the same curative effect as open surgery.
3. Thoracotomy or video-assisted thoracic surgery (VATS) for mediastinal nodes
For Castleman’s tumour in the mediastinum (central chest), surgeons may perform a thoracotomy (open chest) or a less-invasive VATS procedure. Wikipedia+4PMC+4Austin Publishing Group+4 These operations are done to remove nodes that compress lungs, heart, or major vessels, relieve symptoms such as cough and breathlessness, and cure unicentric disease. The choice of approach depends on size, location, and surgeon expertise.
4. Splenectomy
In some multicentric or complex cases with massive splenomegaly or hypersplenism, surgical removal of the spleen can be considered. Wikipedia+4ASHPublications+4PMC+4 This is done when the spleen is destroying blood cells (causing pancytopenia), causing pain, or contributing significantly to disease activity. Splenectomy can quickly improve blood counts and reduce abdominal discomfort, but it increases long-term infection risk, so vaccination and antibiotic precautions are vital.
5. Debulking or palliative surgery
In advanced cases where disease is not fully resectable, debulking surgery may be performed to remove as much tumour mass as safely possible. BioMed Central+4ScienceDirect+4PMC+4 This is done to relieve compression of organs (e.g., bowel obstruction, nerve compression), reduce pain, and improve the effectiveness of radiotherapy or systemic treatments. It is not curative but can greatly improve quality of life.
Prevention strategies
Castleman disease itself cannot always be prevented, especially idiopathic forms. However, you can reduce complications and relapses by:
Strict infection prevention – Hand hygiene, mask use in high-risk settings, and prompt treatment of infections. Haematologica+4Rare Awareness Rare Education Portal+4CDCN+4
Keeping vaccinations up to date – Influenza, pneumococcal, COVID-19, hepatitis, and other vaccines as advised. Wikipedia+4Rare Awareness Rare Education Portal+4CDCN+4
Adhering to HIV antiretroviral therapy if HIV-positive to lower HHV-8-driven MCD risk and relapse. PMC+4PMC+4ScienceDirect+4
Regular follow-up visits with blood tests and scans to catch relapses early. Wiley Online Library+4ASHPublications+4PMC+4
Avoiding unnecessary immunosuppressive drugs unless clearly indicated, to limit infection and malignancy risks. Wikipedia+4Medscape+4PMC+4
Healthy weight, exercise, and non-smoking lifestyle to reduce cardiovascular and thrombotic complications. Wikipedia+4ASHPublications+4Medscape+4
Bone-health protection (vitamin D, calcium, weight-bearing exercise) if on steroids. ASHPublications+4Medscape+4PMC+4
Prompt reporting of new symptoms such as fevers, weight loss, or enlarged nodes to your doctor. Wikipedia+4Mayo Clinic+4Medscape+4
Careful sun and skin protection if on drugs that increase photosensitivity or skin cancer risk. Wikipedia+4e-Century Publishing+4ResearchGate+4
Engaging in clinical trials or registries to receive cutting-edge care and close monitoring. ResearchGate+4CDCN+4ASHPublications+4
When to see a doctor (or go to emergency)
You should seek urgent medical help or contact your Castleman specialist if you have any of the following:
High fever, chills, or sweats, especially with low blood counts or while on biologics/chemotherapy, as this may signal sepsis. Wikipedia+4ASHPublications+4Medscape+4
Rapidly enlarging lymph nodes, new lumps, or swelling that is painful or compressing airway or major vessels. Wikipedia+4Mayo Clinic+4Medscape+4
Severe shortness of breath, chest pain, or sudden cough, which may reflect pulmonary involvement, pulmonary embolism, or heart issues from treatment. Wikipedia+4Medscape+4PMC+4
Uncontrolled bleeding or bruising, suggesting low platelets or coagulation problems. ASHPublications+4ASHPublications+4PMC+4
Severe abdominal pain, swelling, or jaundice, which may indicate spleen rupture, liver involvement, or intestinal complications. Cureus+4ASHPublications+4Medscape+4
New severe headache, confusion, or neurologic symptoms, which can be complications of hypertension, thrombosis, or drug side effects. ASHPublications+4PMC+4Haematologica+4
For routine questions, fatigue, mild weight loss, or minor symptom changes, arrange a non-emergency appointment to review your treatment plan.
What to eat and what to avoid
What to eat
Plenty of colourful fruits and vegetables – Provide antioxidants, vitamins, and fibre that support immune function and gut health. ASHPublications+4Medscape+4PMC+4
Lean protein sources such as fish, eggs, beans, and poultry to rebuild muscle and support blood-cell production. Haematologica+4Medscape+4PMC+4
Healthy fats from olive oil, nuts, seeds, and avocados to provide energy and omega-3s without excessive saturated fat. JCI+4Medscape+4PMC+4
Whole grains (brown rice, oats, whole-wheat bread) for sustained energy and fibre to manage constipation from painkillers or thalidomide-like drugs. JCI+4Medscape+4PMC+4
Adequate fluids (water, herbal teas, broths) to stay hydrated, support kidney function, and help prevent clots and constipation. Journal of Pediatrics+4Medscape+4PMC+4
What to avoid or limit
Highly processed, salty, and sugary foods (fast food, packaged snacks, sodas) that can worsen weight gain, blood pressure, and blood sugar, especially on steroids. ASHPublications+4Medscape+4PMC+4
Very high alcohol intake, which stresses the liver and interacts with many Castleman medications. Wikipedia+4Medscape+4PMC+4
Unpasteurised dairy, raw eggs, and undercooked meats when immunosuppressed, to reduce food-borne infections. Wikipedia+4Rare Awareness Rare Education Portal+4CDCN+4
Grapefruit or Seville orange products with certain drugs (like some chemotherapies or sirolimus) because of CYP3A4 interactions—ask your pharmacist. Wikipedia+4PubMed+4JCI+4
Herbal supplements without medical review, as some may affect liver function, blood clotting, or drug levels. JCI+4PMC+4Haematologica+4
Frequently asked questions (FAQs)
1. Is Castleman’s tumour cancer?
Castleman disease is often called “cancer-like.” It is a lymphoproliferative disorder, not a classical malignant lymphoma, but multicentric forms can behave aggressively and increase the risk of developing lymphoma later. Haematologica+4Wikipedia+4Wikipedia+4
2. What is the difference between unicentric and multicentric Castleman disease?
Unicentric Castleman disease affects a single lymph-node area and is usually cured by surgery. Multicentric Castleman disease involves many regions, causes systemic symptoms, and needs systemic treatments such as siltuximab, rituximab, or chemotherapy. Medscape+4Wikipedia+4Wikipedia+4
3. What does “idiopathic multicentric Castleman disease (iMCD)” mean?
“Idiopathic” means the cause is unknown and not clearly linked to viruses like HHV-8 or HIV. iMCD patients are usually HIV- and HHV-8-negative and are treated mainly with IL-6-targeted drugs such as siltuximab or tocilizumab. Medscape+4Wikipedia+4ASHPublications+4
4. How is Castleman disease diagnosed?
Doctors combine symptoms, blood tests, imaging, and especially lymph-node biopsy with expert pathology review. Criteria also require exclusion of other causes like lymphoma, autoimmune disease, and infections. Medscape+4ASHPublications+4PMC+4
5. What is the prognosis?
Unicentric disease generally has an excellent prognosis after complete surgery. Prognosis in multicentric disease varies—from chronic manageable illness to life-threatening—but outcomes have improved significantly with IL-6-targeted therapies and rituximab. ASHPublications+4ASHPublications+4PMC+4
6. Can Castleman disease be cured?
Many patients with unicentric disease are cured by surgery. Multicentric disease is often controlled rather than completely cured, though some patients experience long-term remission with siltuximab, rituximab-based regimens, or newer targeted approaches. JCI+4ScienceDirect+4Austin Publishing Group+4
7. Why is IL-6 so important in Castleman disease?
IL-6 is a cytokine that drives fever, anaemia, high CRP, and B-cell activation. High IL-6 levels are a hallmark of many Castleman cases, and blocking IL-6 or its receptor with siltuximab or tocilizumab often leads to major improvement. Haematologica+3ASHPublications+3PubMed+3
8. What is the role of HHV-8 and HIV?
In HHV-8-positive MCD, the virus infects B cells and produces viral IL-6–like proteins, especially in people living with HIV. This explains why rituximab, antivirals, and effective HIV therapy are central in this subtype. MDPI+4PMC+4Rare Awareness Rare Education Portal+4
9. Will I need treatment forever?
Some patients require long-term or even indefinite biologic therapy to keep iMCD under control, while others can taper treatment after stable remission. Decisions depend on response, side effects, and relapse risk and are made by your specialist team. Wiley Online Library+4ASHPublications+4PMC+4
10. Can Castleman disease come back after surgery or treatment?
Yes. Unicentric disease rarely recurs after complete excision, but follow-up is still needed. Multicentric disease often relapses and may require changes in therapy, such as switching from IL-6 blockers to sirolimus or adding chemotherapy. SpringerLink+4ScienceDirect+4Austin Publishing Group+4
11. Is pregnancy possible with Castleman disease?
Some patients have successful pregnancies, but careful planning is needed because many treatments (thalidomide, lenalidomide, some chemotherapies) are highly teratogenic. Pre-pregnancy counselling and coordination between haematology and obstetrics are essential. Haematologica+4e-Century Publishing+4ResearchGate+4
12. Are there lifestyle changes that really make a difference?
Yes. Not smoking, limiting alcohol, staying physically active, eating a nutrient-dense diet, and managing stress can all help reduce complications, maintain strength, and improve quality of life, even though they do not replace medical treatment. ASHPublications+4Medscape+4PMC+4
13. How rare is Castleman disease?
Idiopathic multicentric Castleman disease is estimated at roughly 1500–1800 new cases per year in the United States, and unicentric disease is somewhat more common. Medscape+4Wikipedia+4Wikipedia+4
14. Where can I find reliable information and support?
Major academic centres (such as Mayo Clinic) and the Castleman Disease Collaborative Network (CDCN) provide trustworthy patient guides, treatment updates, and research-trial information, as well as community support. PMC+4CDCN+4Mayo Clinic+4
15. Is all this information enough to choose my treatment alone?
No. Castleman disease is complex and highly individual. This article provides an educational overview, but treatment must be designed and adjusted by experienced specialists who know your full medical history, test results, and preferences. Always discuss any drug, supplement, or lifestyle change with your own doctor before starting it.
Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.
The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members
Last Updated: November 13, 2025.
