Benign angiofollicular hyperplasia is a non-cancer growth of lymph node tissue. It means that the lymph node (a small gland that helps fight infections) becomes bigger and more active than normal, but the cells do not behave like cancer. Under the microscope, doctors see many small round structures called follicles and an increased number of tiny blood vessels (angio = blood vessel, follicular = follicles, hyperplasia = overgrowth). This pattern is why the disease is called “angio-follicular hyper-plasia.” ScienceDirect+1
Benign angiofollicular hyperplasia is another name for Castleman disease. It is a rare disease where the lymph nodes grow too much and become “overactive.” Doctors call it a lymphoproliferative disorder, meaning some immune cells in the lymph nodes multiply more than normal and produce many inflammatory proteins such as interleukin-6 (IL-6).GARD Information Center+2Cancer.gov+2
There are two main patterns:
Unicentric Castleman disease (UCD) – only one lymph node (or one area) is affected. Surgery can often cure this.PMC+1
Multicentric Castleman disease (MCD) – many lymph node areas are involved. It can cause fever, weight loss, night sweats, anemia, and other serious problems, and needs long-term medical treatment.PMC+1
Today, doctors usually consider benign angiofollicular hyperplasia to be part of a group of diseases called Castleman disease, especially the localized or “unicentric” type, where only one lymph node area is involved. These enlarged lymph nodes can be in the chest, neck, abdomen, or other parts of the body. The condition is rare, and many people have no symptoms; it is often found by chance on a scan or during surgery for another reason. Wikipedia+2Wikipedia+2
On a microscopic level, the most common pattern in benign angiofollicular hyperplasia is called the hyaline-vascular type. The follicles are small and shrunken, the outer “mantle” of lymphocytes forms “onion-skin” layers, and small blood vessels run into the center like a “lollipop” sign. These findings show an abnormal but benign reaction of the immune system inside the lymph node. Cancer.gov+2PMC+2
Other names
Benign angiofollicular hyperplasia has several other names used in older and newer medical writings. These names all describe the same or very closely related conditions:
Angiofollicular lymph node hyperplasia – focuses on the lymph node itself. Cancer.gov+1
Giant lymph node hyperplasia – used because the affected lymph node can become very large. Cancer.gov+1
Lymphoid hamartoma or lymph nodal hamartoma – “hamartoma” means a benign overgrowth of normal tissue in the wrong shape. path.upmc.edu+1
Angiofollicular hyperplasia – a shorter form often used in case reports. Cancer Treatment Reviews+1
Castleman disease (especially unicentric Castleman disease) – this is the modern umbrella name used for most cases with this pattern. Wikipedia+2Wikipedia+2
These different terms can be confusing, but they all point to a rare benign lymph-node overgrowth with a characteristic microscopic look.
Types
Doctors describe benign angiofollicular hyperplasia (Castleman-type disease) in two main ways: by how many lymph node areas are involved and by what the lymph node looks like under the microscope.
Types by how many lymph node areas are involved
1. Unicentric (localized) type
In unicentric disease, only one lymph node or one group of lymph nodes in a single area (for example, one region in the chest or neck) is enlarged. This is the most common and usually the “benign” form. Many patients have no general (systemic) symptoms, and surgical removal of the enlarged node often cures the problem. Wikipedia+2CDCN+2
2. Multicentric (systemic) type
In multicentric disease, many lymph node areas in different parts of the body are enlarged at the same time. This pattern is often linked to strong inflammation and symptoms such as fever, weight loss, night sweats, and enlarged liver or spleen. It can be associated with infections such as human herpesvirus-8 (HHV-8) and with immune problems. Multicentric disease behaves more like a serious systemic illness and may not be purely “benign,” even though the growth still comes from lymph node hyperplasia rather than typical malignant lymphoma. Wikipedia+2CDCN+2
Types by microscopic (histologic) pattern
3. Hyaline-vascular type
This is the classic pattern for benign angiofollicular hyperplasia. The follicles are small and shrunken, with a glassy (“hyaline”) appearance in the center and many small blood vessels entering the follicles. The surrounding lymphocytes form “onion-skin” layers. This type is most often found in unicentric disease and usually has few or no systemic symptoms. PMC+2path.upmc.edu+2
4. Plasma cell type
In the plasma cell type, the follicles are more enlarged, and there are many plasma cells (antibody-producing cells) in the tissue between follicles. This type is more often linked to systemic symptoms such as fever, anemia, and high inflammatory markers, and it is more frequent in multicentric Castleman disease. PMC+2Wikipedia+2
5. Mixed type
Some lymph nodes show features of both hyaline-vascular and plasma cell types. In these cases, doctors call it a mixed pattern. The clinical behavior depends on how extensive the plasma cell changes and systemic symptoms are. ASHPublications+1
6. Hypervascular variant
Recent literature also describes a hypervascular pattern, with very prominent blood vessels and regressed follicles. This is especially mentioned in idiopathic multicentric Castleman disease and overlaps with the other patterns, showing how variable the disease can look under the microscope. Wikipedia+2CDCN+2
Causes and contributing factors
The exact cause of benign angiofollicular hyperplasia, especially in unicentric cases, is often unknown. Many cases are called “idiopathic,” meaning “cause not known.” However, research has suggested several possible triggers and risk factors. In simple English, we will treat these as “possible causes or contributors,” understanding that for many patients, no clear cause is proven. CDCN+1
1. Local benign lymph node tumor-like change
Unicentric disease may behave like a benign tumor of the lymph node stroma (supporting cells), in which a single node starts growing abnormally but non-cancerously, causing angiofollicular hyperplasia. This is one leading theory for localized disease. CDCN+1
2. Over-production of interleukin-6 (IL-6)
Interleukin-6 (IL-6) is a signaling protein (cytokine) that controls inflammation and immune responses. Many patients with Castleman disease have very high IL-6 levels, which can drive lymph node overgrowth and systemic symptoms like fever and weight loss. PMC+2CDCN+2
3. Infection with human herpesvirus-8 (HHV-8)
In multicentric cases, especially in people with HIV, HHV-8 infection is a well-proven trigger. The virus produces its own version of IL-6, which strongly stimulates lymph node cells and causes overgrowth and inflammation. This viral effect is less clear in strictly benign, localized cases but is important overall in this disease family. PMC+2PMC+2
4. HIV infection and immune weakness
People living with HIV can develop HHV-8-associated multicentric Castleman disease. The weakened immune system allows the virus to act more strongly and promotes uncontrolled cytokine release and lymph node hyperplasia. bloodresearch.or.kr+1
5. Other chronic viral infections (e.g., EBV)
Some reports suggest a role for other long-lasting viral infections, such as Epstein–Barr virus (EBV), which can disturb immune regulation and promote abnormal lymphoid growth, though the evidence is less strong than for HHV-8. eurobloodnet.eu+1
6. Genetic susceptibility in immune or stromal cells
Research suggests that specific genetic changes in lymph node stromal or immune cells might make them more likely to grow abnormally and respond strongly to cytokines. This idea is still being studied but may explain why only certain people develop the disease. CDCN+1
7. Autoimmune-like immune dysregulation
Castleman-type diseases share some features with autoimmune diseases, such as chronic inflammation and abnormal antibody production. In some patients, the immune system may attack or overstimulate its own lymph node tissue in a chronic way, leading to hyperplasia. ASHPublications+1
8. Association with POEMS syndrome
POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes) is sometimes associated with multicentric Castleman disease. The abnormal plasma cells and cytokines in POEMS may drive angiofollicular hyperplasia in lymph nodes. CDCN+1
9. Association with TAFRO syndrome
TAFRO stands for Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis, and Organomegaly. It is a particular subtype of idiopathic multicentric Castleman disease with aggressive inflammation. The strong cytokine storm in TAFRO may cause the angiofollicular pattern in multiple lymph nodes. CDCN+1
10. Chronic low-grade infections (bacteria or other organisms)
Some researchers suspect that long-term, low-grade infections, even if not clearly identified, might stimulate lymph nodes for many years and gradually cause overgrowth. Evidence is limited, but this is one proposed mechanism. Cancer Treatment Reviews+1
11. Chronic inflammatory states and metabolic stress
Ongoing inflammation from obesity, chronic liver disease, or other inflammatory conditions can increase cytokine levels, including IL-6, and may indirectly encourage lymph node overactivity and hyperplasia. CDCN+1
12. Immune reconstitution after HIV treatment
Cases have been reported where Castleman disease appears or changes during strong HIV treatment (antiretroviral therapy). Rapid changes in immune function may unmask or trigger abnormal lymph node responses in patients with underlying HHV-8 infection. Nature+1
13. Paraneoplastic phenomena (response to another tumor)
In some patients, angiofollicular hyperplasia may occur as an immune reaction to another tumor somewhere else in the body (paraneoplastic response), although this is less common and still under investigation. Cancer Treatment Reviews+1
14. Environmental or toxic exposures (theoretical)
Certain toxins or environmental exposures that chronically stimulate the immune system could, in theory, promote lymph node hyperplasia. Clear proof is limited, but this is sometimes discussed as a contributing possibility. Cancer Treatment Reviews+1
15. Hormonal and endocrine factors
Some patients with Castleman disease have endocrine problems, and POEMS syndrome includes hormone abnormalities. Hormonal imbalances may influence immune cell growth and cytokine production, indirectly contributing to hyperplasia. CDCN+1
16. Clonal expansion of B cells or plasma cells
In some cases, there is evidence of clonal (single-cell origin) expansion of B cells or plasma cells, which can produce excess antibodies and cytokines, pushing the lymph node into an angiofollicular pattern without becoming full malignant lymphoma. PMC+2ASHPublications+2
17. Abnormal follicular dendritic cells
Follicular dendritic cells help arrange B cells inside lymph node follicles. Their overgrowth or dysplasia has been described in Castleman disease, and may be a primary driver of the abnormal follicle and vessel structure. PMC+2CDCN+2
18. Age-related immune changes
Castleman-type diseases can appear in adults of various ages. Changes in immune regulation that occur with aging may make lymph nodes more likely to respond abnormally to triggers, though this is still a theoretical factor. Cancer Treatment Reviews+1
19. Genetic or epigenetic changes in cytokine pathways
Alterations in genes that control IL-6 or related cytokine pathways may make a person’s immune system more prone to excessive activation. This could explain why some people develop the disease after a common infection while most do not. PMC+2ScienceDirect+2
20. Idiopathic (unknown cause)
In many patients, especially those with a single, localized lymph node, no clear infection, genetic change, or associated syndrome is found. These cases are called idiopathic. Even though the cause is unknown, the condition can still be diagnosed based on imaging and biopsy findings. CDCN+2ASHPublications+2
Symptoms and clinical features
Not everyone with benign angiofollicular hyperplasia has symptoms. Unicentric disease is often silent. When symptoms do occur, they may come from the enlarged lymph node pressing on nearby structures or from systemic inflammation (more common in multicentric disease). PMC+2Mayo Clinic+2
1. Painless lump or mass
The most typical sign is a painless, firm lump in the neck, armpit, chest, or abdomen. This lump is an enlarged lymph node. It often grows slowly and may be found by a doctor during a routine exam or by imaging done for another reason. Wikipedia+2Jornal de Pneumologia+2
2. Chest pain or tightness
If the enlarged lymph node is in the chest (mediastinum), it can press on nearby structures such as the lungs or blood vessels, causing chest pain, tightness, or discomfort. This pain is usually dull and related to the size and location of the mass. PMC+2PMC+2
3. Cough or shortness of breath
A chest mass may also compress the airways, leading to a chronic cough or feeling short of breath, especially when lying flat or exercising. These symptoms can look like asthma or other chest problems, which is why imaging is important. PMC+2PMC+2
4. Difficulty swallowing (dysphagia)
If the lymph node is enlarged in the middle chest or near the esophagus, it can press on the food pipe and make swallowing solid food uncomfortable or difficult. Some patients may feel as if food gets stuck halfway down. PMC+2Radiology Journal+2
5. Abdominal pain or fullness
Enlarged lymph nodes in the abdomen or retroperitoneum can cause a feeling of fullness, dull pain, or pressure. Sometimes this can mimic other abdominal problems, and patients may feel bloated even after eating small amounts. PMC+2ScienceDirect+2
6. Early fullness after meals (early satiety)
When a large mass or enlarged spleen presses on the stomach, patients may feel full quickly when eating, which can lead to weight loss over time. Wikipedia+1
7. Enlarged abdomen from liver or spleen enlargement
In multicentric disease, the liver and spleen can become enlarged (organomegaly). Patients may notice a swollen belly, discomfort under the ribs, or a doctor may feel large organs on exam. Wikipedia+2CDCN+2
8. Fever
Many patients with systemic forms have recurrent or persistent fevers due to high levels of IL-6 and other inflammatory cytokines. The fever often comes with night sweats and may be mistaken for infection or lymphoma. PMC+2ASHPublications+2
9. Night sweats
Soaking night sweats are a common inflammatory symptom. Patients may wake up with drenched clothes or bedding, similar to what is seen in lymphomas, which is why a biopsy is needed to separate these conditions. Wikipedia+2Mayo Clinic+2
10. Unintentional weight loss
Ongoing inflammation and reduced appetite can lead to losing weight without trying. This can be subtle or significant, depending on disease activity. Mayo Clinic+1
11. Fatigue and weakness
Fatigue is very common. It comes from chronic inflammation, poor sleep from night sweats, and sometimes from anemia (low red blood cell count). Patients may find it hard to carry out daily tasks or feel tired all day. PMC+2ASHPublications+2
12. Recurrent infections
If the immune system is disturbed, or if the spleen or bone marrow function is affected, patients might have more frequent infections such as respiratory or skin infections. This can be related both to the disease and to associated conditions like HIV. bloodresearch.or.kr+1
13. Anemia-related symptoms (pale skin, dizziness, short breath)
Many patients with multicentric disease develop anemia. They may look pale, feel dizzy or light-headed, and become short of breath with mild activity. Lab tests often confirm low hemoglobin. PMC+2ASHPublications+2
14. Numbness or tingling in hands and feet
In patients with POEMS or other associated neuropathies, nerve damage can cause numbness, tingling, burning pain, or weakness in the limbs. This neuropathy is related to abnormal antibodies and cytokines affecting nerves. CDCN+2ASHPublications+2
15. Easy bruising or bleeding
If the bone marrow is affected or the spleen is enlarged and trapping platelets, patients may bruise easily, have frequent nosebleeds, or notice longer bleeding from cuts. This reflects low platelet counts or clotting problems. CDCN+2ASHPublications+2
Diagnostic tests
Diagnosis of benign angiofollicular hyperplasia always requires a combination of history, physical exam, laboratory tests, imaging, and lymph node biopsy. No single blood test can confirm it. A key goal is to distinguish it from lymphoma, infection, or other tumors. CDCN+2ASHPublications+2
Physical examination tests
1. General physical examination and vital signs (Physical exam)
The doctor checks temperature, blood pressure, heart rate, breathing rate, and body weight. They look for fever, weight loss, pale skin, sweating, swelling, and general signs of illness. This gives an overview of how active the disease might be and whether there is systemic inflammation. UpToDate+2Mayo Clinic+2
2. Lymph node examination by inspection and palpation (Physical exam)
The doctor carefully feels (palpates) lymph node areas in the neck, armpits, groin, and above the collarbones. They look for size, consistency (soft or firm), tenderness, and whether nodes are fixed or mobile. In benign angiofollicular hyperplasia, nodes are usually firm, non-tender, and rubbery, and may be solitary or localized. PMC+2Wikipedia+2
3. Abdominal examination for organ enlargement and fluid (Physical exam)
The doctor gently presses and taps the abdomen to feel for enlarged liver, enlarged spleen, deep lymph node masses, or fluid in the abdomen (ascites). Organ enlargement is more common in multicentric disease and can guide further imaging. CDCN+2ASHPublications+2
Manual bedside tests
4. Targeted neck and chest mass palpation with range of motion (Manual exam)
For masses near the neck or chest wall, the doctor palpates while the patient turns the head, raises arms, or takes deep breaths. This manual test helps to judge whether the mass is arising from lymph nodes, muscle, or bone, and whether it moves with breathing (suggesting a mediastinal or lung relation). PMC+2PMC+2
5. Peripheral edema and ascites assessment (Manual exam)
By pressing fingers over the lower legs and ankles (for pitting edema) and tapping the abdomen (for shifting dullness), the doctor checks for fluid build-up. Edema and ascites are part of TAFRO and other severe Castleman-related syndromes, reflecting systemic inflammation and organ involvement. CDCN+1
6. Bedside neurologic screening (Manual muscle and sensory exam)
The doctor checks muscle strength, reflexes, and sensation with simple manual tests, such as pushing against the patient’s hands or feet and testing light touch or vibration. This helps detect neuropathy in patients with POEMS or other associated conditions. CDCN+1
Laboratory and pathological tests
7. Complete blood count (CBC) with differential (Lab test)
The CBC measures red cells, white cells, and platelets. In multicentric disease, anemia, low platelets, or high white cells can be present. The differential shows the types of white cells, which helps exclude leukemia or lymphoma and supports the diagnosis when combined with other findings. Medscape+2CDCN+2
8. Inflammatory markers – ESR and CRP (Lab test)
Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) show the level of inflammation. Many patients with active Castleman-type disease have very high ESR and CRP, reflecting IL-6–driven inflammation. These tests are not specific but help measure disease activity and response to treatment. PMC+2Medscape+2
9. Liver and kidney function tests (Lab test)
Blood tests such as ALT, AST, bilirubin, albumin, creatinine, and urea show whether the liver and kidneys are affected. Abnormal results may occur in severe multicentric disease, TAFRO syndrome, or when enlarged nodes compress surrounding organs or blood vessels. CDCN+2CDCN+2
10. Serum protein electrophoresis and immunoglobulin levels (Lab test)
This test measures the different proteins and antibodies in the blood. Some patients have increased immunoglobulins (polyclonal hypergammaglobulinemia), which shows chronic immune activation. Others may have a monoclonal protein as part of POEMS syndrome. PMC+2CDCN+2
11. HIV and HHV-8 testing (Lab / virology tests)
Because multicentric Castleman disease is strongly linked to HIV infection and HHV-8, tests for these viruses are important. HIV testing is usually done with standard antibody and antigen tests, and HHV-8 can be detected by antibody tests or PCR in blood or tissue. PMC+2Medscape+2
12. Autoimmune and other infection screens (Lab test)
Doctors may order antinuclear antibodies (ANA), hepatitis tests, and other screens to check for autoimmune diseases or infections that can mimic or coexist with Castleman-type disease. These tests help to exclude other causes of lymph node enlargement and inflammation. ASHPublications+1
13. Measurement of IL-6 and related cytokines (Special lab test)
In specialized centers, IL-6 and other cytokines can be measured. Very high IL-6 levels support the diagnosis of Castleman-type disease and help explain systemic symptoms like fever and fatigue. However, this test is not always available and is not required for diagnosis. PMC+2ScienceDirect+2
14. Lymph node excisional biopsy with routine histology (Pathological test – essential)
The key diagnostic test is surgical removal (excision) of the entire affected lymph node and detailed microscopic examination. Pathologists look for characteristic angiofollicular features: small, regressed germinal centers, “onion-skin” mantle zones, and many small blood vessels. Without this biopsy, the diagnosis cannot be confirmed and lymphoma or other diseases cannot be safely excluded. path.upmc.edu+2ASHPublications+2
15. Immunohistochemistry and special stains on lymph node tissue (Pathological test)
Pathologists use special antibody stains to highlight certain cell types and proteins in the lymph node. These tests help distinguish Castleman-type hyperplasia from malignant lymphoma, detect HHV-8 infection in some cases, and classify the histologic subtype. PMC+2path.upmc.edu+2
Electrodiagnostic tests
16. Electrocardiogram (ECG) (Electrodiagnostic test)
An ECG records the electrical activity of the heart. It is not specific for benign angiofollicular hyperplasia, but doctors may use it to check heart function in patients with severe systemic disease, fluid overload, or treatments that can affect the heart. It helps to rule out other causes of symptoms such as chest discomfort or shortness of breath. ASHPublications+1
17. Nerve conduction study and electromyography (NCS/EMG) (Electrodiagnostic test)
In patients with neuropathy (for example, in POEMS syndrome), nerve conduction studies and EMG examine how well the nerves and muscles conduct electrical signals. Abnormal results support the presence of polyneuropathy, an important clue to a Castleman-related systemic disorder. CDCN+1
Imaging tests
18. Ultrasound of lymph nodes and abdomen (Imaging test)
Ultrasound uses sound waves to create images of soft tissues. It can show the size, shape, and internal pattern of enlarged lymph nodes and detect enlarged liver, spleen, or fluid in the abdomen. It is often used as a first imaging step and to guide needle biopsies, although excisional biopsy is usually preferred. PMC+2ScienceDirect+2
19. CT scan of chest, abdomen, and pelvis (Imaging test)
Computed tomography (CT) provides detailed cross-sectional images and is widely used to locate enlarged lymph nodes in the chest, abdomen, and pelvis. In unicentric disease, CT often shows a single well-defined, homogeneously enhancing mass. CT also helps determine if disease is unicentric or multicentric, which is crucial for planning treatment. PMC+2Radiology Journal+2
20. MRI and PET-CT scans (Imaging test)
Magnetic resonance imaging (MRI) can provide detailed images of soft tissue masses, especially in complex areas like the neck or spine. Positron emission tomography combined with CT (PET-CT) shows both anatomy and metabolic activity; Castleman lesions often show moderate to intense uptake of FDG. PET-CT helps to see how many lymph node areas are involved and to choose the best node for biopsy. ejcrim.com+3PMC+3CDCN+3
Non-pharmacological treatments
1. Careful monitoring and “watchful waiting”
In some patients with unicentric disease who have mild or no symptoms, doctors may choose “watchful waiting” after diagnosis or surgery. The purpose is to avoid unnecessary treatment when the disease is quiet. The doctor checks the patient regularly with physical exams, blood tests, and sometimes scans. If symptoms appear (fever, weight loss, new swollen nodes), the plan changes to active treatment. The mechanism is simple: early detection of change. By watching trends in blood counts and inflammation markers (like CRP), the doctor can act quickly if the disease becomes more active.
2. Patient education and counseling
Education means explaining the disease in simple words, including the difference between unicentric and multicentric forms, and possible complications like infections or lymphoma.Cancer.gov+1
The purpose is to reduce fear, improve understanding, and help the patient take part in decisions. The mechanism is psychological: when patients understand why they need follow-up, vaccines, or certain drugs, they are more likely to follow the plan and recognise warning signs early. Counseling can also help manage anxiety and depression that often appear with chronic rare diseases.
3. Nutritional counseling and individualized meal planning
A dietitian can design a balanced anti-inflammatory diet rich in fruits, vegetables, whole grains, and lean proteins. The purpose is to support the immune system, maintain weight, and reduce fatigue. Mechanistically, good nutrition provides vitamins, minerals, and antioxidants that help red blood cells, white blood cells, and the liver work better. It also helps the body tolerate surgery, chemotherapy, or antibody treatments. For patients with weight loss or low appetite, high-calorie and high-protein foods and oral nutrition drinks may be used.
4. Infection prevention and hygiene measures
Because Castleman disease and its treatments can weaken the immune system, infection prevention is very important. The purpose is to reduce the risk of serious infections, such as pneumonia or sepsis. Mechanisms include simple but effective habits: regular handwashing, mask use in crowded places during outbreaks, safe food handling, and avoiding close contact with people who are sick. For some patients, the treating doctor may also recommend prophylactic (preventive) antibiotics or antiviral drugs, especially when strong immunosuppressive treatment is used.
5. Vaccination programs
Doctors often recommend up-to-date vaccines for flu, COVID-19, pneumococcus, hepatitis B, and others, when appropriate. The purpose is to prevent infections that can be very dangerous in an immunocompromised patient. The mechanism is immune priming: vaccines teach the immune system to recognise specific germs so it can respond faster and stronger. Vaccination timing is important – ideally before starting powerful immunosuppressive therapies, because such drugs can reduce the vaccine response. The exact schedule is chosen by the hematologist or infectious disease specialist.
6. Gentle physical activity and exercise
Regular light to moderate exercise, such as walking, stretching, yoga, or cycling at a comfortable pace, can improve energy and mood. The purpose is to fight fatigue, preserve muscle strength, and support heart and lung function. Mechanistically, movement improves circulation and oxygen delivery, stimulates muscle and bone, and releases endorphins in the brain, which can reduce pain perception and improve sleep. Exercise plans must be adapted to the patient’s condition; on days with fever or very low blood counts, rest may be more appropriate.
7. Physiotherapy and rehabilitation after surgery or during long-term therapy
When patients undergo surgery to remove lymph nodes or receive long-term systemic treatment, they may develop stiffness, weakness, or limited mobility. Physiotherapy aims to restore movement, prevent contractures, and reduce pain. Mechanisms include guided exercises, range-of-motion training, manual therapy, and posture correction. A trained physiotherapist can also teach safe ways to move and lift, reducing strain on the surgical area and spine. This kind of therapy supports overall recovery and independence.
8. Pain management with non-drug techniques
Some patients have pain from enlarged lymph nodes, surgery, or inflammatory flares. Non-drug methods such as heat or cold packs, relaxation breathing, mindfulness, gentle massage (when safe), and TENS (transcutaneous electrical nerve stimulation) can help. The purpose is to reduce pain and lower the need for high doses of pain medicines. Mechanistically, these approaches change how nerves send pain signals and how the brain interprets them. They can also reduce muscle tension and help the patient feel more in control of symptoms.
9. Stress management and psychological therapies
Living with a rare, chronic disease often causes stress, fear of relapse, and worries about work and family. Psychological therapies such as cognitive-behavioral therapy (CBT), supportive counseling, or group therapy can help. The purpose is to improve emotional well-being and coping skills. Mechanisms include teaching patients to recognize negative thought patterns, build healthy routines, and use problem-solving strategies. Lower stress levels can also improve sleep, appetite, and possibly immune balance.
10. Social work support and patient support groups
Social workers help patients deal with work, insurance, disability benefits, and travel to specialist centers. The purpose is to reduce the practical burden of treatment. Mechanistically, when a social worker coordinates services, patients and families have less stress and can focus more on health. Patient support groups, including online communities for Castleman disease, allow patients to share experience, tips, and emotional support, which reduces feelings of isolation.
11. Lymphedema management and compression therapy
Enlarged lymph nodes or surgery may disturb lymph drainage and cause swelling in an arm, leg, or nearby area. Lymphedema therapy includes elevation, compression garments, manual lymph drainage, and skin care. The purpose is to limit swelling, reduce discomfort, and prevent skin infections. Mechanistically, compression and massage push lymph fluid back into the central circulation and prevent buildup. Good skin care reduces cracks and entry points for bacteria.
12. Sleep hygiene and fatigue management
Fatigue is common, especially in multicentric disease. Simple sleep hygiene habits can help: regular sleep time, limiting screen exposure before bed, quiet and dark room, and avoiding large meals and caffeine late at night. The purpose is to improve sleep quality and daytime energy. The mechanism is regulation of the body’s internal clock and hormones like melatonin and cortisol. Fatigue management also includes pacing activities and planning rest breaks.
13. Smoking cessation
If the patient smokes, stopping is a key non-pharmacological measure. The purpose is to protect the heart, lungs, and immune system, especially during strong systemic therapy. Mechanistically, stopping smoking reduces chronic inflammation, improves oxygen delivery, and decreases the risk of infections, blood clots, and secondary cancers. Counseling, nicotine replacement, and support groups may be used as tools to help the patient quit.
14. Limiting alcohol and other liver toxins
Many drugs used for Castleman disease, including chemotherapy and some biologics, can stress the liver. Excess alcohol adds extra damage. The purpose of limiting alcohol is to protect the liver and reduce side-effect risk. Mechanistically, avoiding alcohol lowers oxidative stress and inflammation in liver cells. Patients should also inform doctors about herbal products or over-the-counter medicines that may harm the liver so these can be reviewed.
15. Weight management and metabolic health
Being underweight can worsen fatigue and healing, while severe obesity can make surgery and anesthesia risky and increase inflammation. The purpose of weight management is to reach a healthy, stable weight. Mechanisms include balanced intake of calories and nutrients, regular movement, and monitoring of blood sugar and cholesterol. Good metabolic health supports better response to treatment and reduces cardiovascular risk in the long term.
16. Coordination of care in a specialized center
Because Castleman disease is rare and complex, many guidelines recommend referral to a specialist center with experience in the disease.PMC+2BSH+2
The purpose is to ensure the patient receives modern, evidence-based care and has access to clinical trials if needed. Mechanistically, a multidisciplinary team (hematologist, surgeon, radiologist, pathologist, infectious disease specialist, dietitian, and psychologist) shares information and decides the best plan together.
17. Radiation therapy (as a local non-drug treatment)
Radiation is not a medicine, but a local treatment using high-energy rays to shrink lymph nodes. It is sometimes used when surgery for unicentric disease is not possible or as a second-line option.Medscape+2BSH+2
The purpose is to control the local mass, reduce pain or compression, and avoid more invasive surgery. Mechanistically, radiation damages the DNA of rapidly dividing cells in the affected lymph node, causing them to die over time.
18. Palliative care and symptom-focused support
Palliative care is not only for end of life. It means expert care aimed at relieving symptoms such as pain, breathlessness, and anxiety at any stage of serious disease. The purpose is to improve quality of life. Mechanisms include careful adjustment of pain medicines, non-drug therapies, emotional support, and family counseling. Palliative specialists work with the hematology team and do not replace disease-directed treatment.
19. Rehabilitation for cognitive and occupational issues
Long-term illness, repeated hospital stays, and strong drugs can affect attention, memory, and work ability. Occupational therapists and neuropsychologists can assess these problems and design exercises to improve function or suggest work adaptations. The purpose is to help the patient return to daily activities, work, or study as much as safely possible. Mechanistically, repeated training of thinking and planning skills helps the brain adapt and build new pathways.
20. Regular cancer surveillance
People with Castleman disease have a higher risk of certain cancers such as lymphoma.Cancer.gov+1
The purpose of surveillance is to detect malignant change early. Mechanisms include scheduled clinical exams, blood tests, and imaging, and sometimes bone marrow exams when indicated. Early detection allows quicker, targeted treatment and may improve survival.
Drug treatments
Important safety note: The drugs below are used only under close supervision by hematology/oncology specialists. Doses are general from published guidelines and FDA labels but must be individualized. Never start, stop, or change any of these medicines without your doctor.
1. Siltuximab (SYLVANT®)
Siltuximab is a monoclonal antibody that directly binds interleukin-6 (IL-6), a key inflammatory protein in multicentric Castleman disease. It is the only drug specifically approved by the U.S. FDA for idiopathic multicentric Castleman disease (iMCD) in HIV-negative and HHV-8-negative adults.FDA Access Data+2FDA Access Data+2
Typical dosing from the FDA label is 11 mg/kg by intravenous infusion every 3 weeks, continued until treatment failure. The purpose is to reduce symptoms, shrink enlarged nodes, and improve anemia by blocking IL-6–driven inflammation. Side effects can include infections, low white blood cell counts, infusion reactions, high lipids, and liver test changes. Patients need regular blood tests and infection monitoring.
2. Tocilizumab
Tocilizumab is an antibody that blocks the IL-6 receptor rather than IL-6 itself. It is FDA-approved for rheumatoid arthritis and some other conditions, but used off-label for Castleman disease, especially in some idiopathic MCD cases where siltuximab is not available.PMC+1
Doses in reports are usually 8 mg/kg IV every 2–4 weeks or subcutaneous regimens, adjusted by the specialist. The purpose is similar to siltuximab: suppress IL-6 signaling to reduce fever, inflammation, and lymph node enlargement. Side effects include infection risk, liver enzyme elevation, high cholesterol, and injection or infusion reactions.
3. Rituximab
Rituximab is an anti-CD20 monoclonal antibody that targets B cells. It is widely used for lymphomas and autoimmune diseases and is a key drug for HHV-8–associated multicentric Castleman disease and some other cases.PMC+2ScienceDirect+2
Common regimens are 375 mg/m² IV once weekly for four weeks or in combination with chemotherapy (e.g., R-CHOP). The purpose is to reduce abnormal B-cell and plasma-cell activity and lower production of inflammatory cytokines. Side effects include infusion reactions, infections, reactivation of hepatitis B, low blood counts, and rare progressive multifocal leukoencephalopathy (PML).
4. Glucocorticoids (e.g., Prednisone)
Prednisone and similar steroids (methylprednisolone, dexamethasone) are often used as initial or adjunct therapy to quickly control inflammation and symptoms.PMC+1
Typical doses vary (for example, 0.5–1 mg/kg/day orally, sometimes pulsed high-dose IV in severe flares), with gradual tapering. The purpose is to reduce fever, night sweats, joint pain, and lymph node size by broadly suppressing immune activity. Side effects can include high blood sugar, weight gain, mood changes, osteoporosis, stomach ulcers, and infection risk, especially with long-term use.
5. CHOP-like chemotherapy (Cyclophosphamide, Doxorubicin, Vincristine, Prednisone)
In aggressive or refractory multicentric disease, or when lymphoma cannot be excluded, doctors may use CHOP or similar regimens.PMC+2ScienceDirect+2
These are given in cycles every 3 weeks. The purpose is to kill rapidly dividing lymphoid cells and gain disease control. Mechanistically, each drug damages cell DNA or cell division in a different way. Side effects include hair loss, nausea, low blood counts, infection risk, heart damage (doxorubicin), nerve damage (vincristine), and infertility; therefore, such regimens are reserved for selected cases.
6. Cyclophosphamide (single or in combinations)
Cyclophosphamide is an alkylating agent used in many lymphoma and autoimmune regimens. In Castleman disease it can be given orally or IV, alone or with steroids or rituximab, especially when other options are limited. The purpose is to suppress overactive lymphoid tissue and plasma cells. Side effects include bone marrow suppression, nausea, hair loss, and bladder irritation; adequate hydration and sometimes protective agents (mesna) are used.
7. Etoposide
Etoposide is a topoisomerase inhibitor that prevents cancer cells from dividing. It is sometimes used in combination regimens for severe or relapsing Castleman disease or associated lymphomas. The purpose is to enhance cytotoxic control of the abnormal lymphoid proliferation. Side effects include low blood counts, hair loss, nausea, and risk of secondary leukemias with high cumulative doses.
8. Doxorubicin (including liposomal forms)
Doxorubicin is an anthracycline that intercalates into DNA and generates free radicals, leading to cell death. It is part of CHOP-type regimens used in some MCD patients with very active disease or overlapping lymphoma. The purpose is to deliver strong anti-tumor activity. Because of the risk of heart damage, total lifetime dose is limited; liposomal formulations may reduce some toxicities. Patients need heart function monitoring.
9. Vincristine
Vincristine is a vinca alkaloid that blocks microtubules and stops cell division. In Castleman disease it is mainly used as part of CHOP or similar combinations. The purpose is synergistic killing of pathologic lymphoid cells. Its main side effect is peripheral neuropathy (numbness, tingling, weakness), which requires close monitoring. Constipation and low blood counts can also occur.
10. Thalidomide
Thalidomide has anti-angiogenic and immunomodulatory effects. It has been used off-label in some refractory Castleman cases, often combined with steroids.PMC+1
The purpose is to reduce abnormal blood vessel growth and cytokine production in affected nodes. Side effects include teratogenicity (severe birth defects), neuropathy, drowsiness, constipation, and blood clots; strict pregnancy prevention programs are required.
11. Lenalidomide
Lenalidomide is a newer immunomodulatory drug related to thalidomide, with different side-effect patterns. It has been reported in small series as a possible option in difficult iMCD cases. Mechanistically, it affects immune cells and cytokine production and has anti-angiogenic effects. Side effects include low blood counts, blood clots, rash, and fatigue. It is also highly regulated because of teratogenic risk.
12. Sirolimus (Rapamycin)
Sirolimus is an mTOR inhibitor used to prevent transplant rejection and treat some cancers. Case reports suggest it can help some patients with refractory iMCD, possibly by damping overactive immune and growth signaling pathways. The purpose is to reduce lymph node growth and inflammation when IL-6 or B-cell–directed therapies fail. Side effects include high cholesterol, mouth sores, delayed wound healing, and infection risk; blood levels must be monitored.
13. Bortezomib
Bortezomib is a proteasome inhibitor used in multiple myeloma. In Castleman disease, it has been tried in selected severe cases, particularly when plasma-cell clones are prominent. The purpose is to induce apoptosis of abnormal plasma cells and reduce antibody and cytokine production. Side effects include peripheral neuropathy, low blood counts, and gastrointestinal upset.
14. Ganciclovir
Ganciclovir is an antiviral that targets herpesviruses. In HHV-8–associated MCD, ganciclovir or its oral prodrug valganciclovir may be used alongside rituximab and other therapies to reduce viral replication.PMC+1
The purpose is to control the virus that drives part of the disease process. Side effects include bone marrow suppression and kidney toxicity, so dosing must be adjusted and blood counts monitored.
15. Valganciclovir
Valganciclovir is the oral form of ganciclovir, used for ongoing suppression of HHV-8 in some settings. The mechanism and side effects are similar but the oral route makes long-term use easier. The purpose is to maintain viral control after initial IV therapy and reduce relapse risk, always under infectious disease specialist guidance.
16. Interferon-alpha
Interferon-alpha is an immune signaling protein used as a drug for some viral infections and cancers. In a few Castleman disease reports, it has been used for its antiviral and immune-modulating actions. The purpose is to enhance antiviral immune responses and suppress abnormal lymphoid proliferation. Side effects are common and include flu-like symptoms, depression, thyroid dysfunction, and blood count changes, so careful monitoring is essential.
17. Intravenous immunoglobulin (IVIG)
IVIG is a pooled antibody product given intravenously. In Castleman patients with recurrent infections, low immunoglobulin levels, or certain autoimmune complications, IVIG may be used. The purpose is to support humoral immunity and reduce infection frequency. Mechanistically, it provides ready-made antibodies and modulates immune responses. Side effects include headache, infusion reactions, kidney strain, and very rare blood clots.
18. Prophylactic antibiotics (e.g., trimethoprim-sulfamethoxazole)
When patients are on strong immunosuppressive therapy or high-dose steroids, doctors sometimes prescribe low-dose antibiotics to prevent specific infections such as Pneumocystis pneumonia. The purpose is to prevent life-threatening opportunistic infections. Side effects depend on the drug and can include allergy, rash, kidney issues, or changes in blood counts, so the benefit–risk balance is considered carefully.
19. Erythropoiesis-stimulating agents (e.g., Epoetin alfa)
Castleman disease can cause anemia due to inflammation and bone-marrow effects. In some cases, especially when chemotherapy is used, erythropoiesis-stimulating agents (ESAs) may be considered to support red blood cell production. The purpose is to reduce transfusion need and improve fatigue. Side effects include high blood pressure, clot risk, and rare pure red cell aplasia; therefore, they are used under strict guidelines.
20. Thrombopoietin receptor agonists (e.g., Eltrombopag)
If platelet counts are very low due to marrow suppression or treatment, thrombopoietin receptor agonists may be used in selected cases. They stimulate platelet production in the bone marrow. The purpose is to decrease bleeding risk and support ongoing therapy. Side effects include liver enzyme elevations, clotting risk, and possible marrow fibrosis with long-term use, so careful monitoring is needed.
Dietary molecular supplements
Evidence for supplements specifically in Castleman disease is limited. Most data come from general immune or cancer-support research. Supplements can also interact with drugs. Always ask your doctor before starting any of them.
1. Vitamin D
Vitamin D is important for bone health and immune regulation. Low vitamin D levels are common in chronic illness. Supplementation (often 800–2000 IU/day, but individualized) may support bone strength, especially when steroids are used, and may modulate immune responses. Mechanistically, vitamin D receptors on immune cells influence how they respond to infection and inflammation. Excess doses, however, can cause high calcium and kidney problems, so blood levels should be checked.
2. Omega-3 fatty acids (fish oil)
Omega-3 fatty acids (EPA and DHA) have anti-inflammatory effects. Typical supplemental doses are 1–2 grams of combined EPA/DHA per day, though this must be tailored. Mechanistically, omega-3s are incorporated into cell membranes and lead to less inflammatory eicosanoids. They may slightly reduce inflammatory markers and support heart health. Side effects can include a fishy aftertaste and, at higher doses, increased bleeding tendency, especially with blood-thinning drugs.
3. Curcumin (from turmeric)
Curcumin is a plant compound with antioxidant and anti-inflammatory properties. Small studies in other diseases show that doses of 500–1500 mg/day (often divided) can reduce inflammatory markers. Mechanistically, curcumin interacts with NF-κB and other signaling pathways that control cytokine production. However, its absorption is low, and many products combine it with piperine or special formulations. It can interact with blood thinners and chemotherapy, so medical advice is essential.
4. Probiotics
Probiotics are “good bacteria” taken as capsules or in fermented foods. Typical doses vary widely (e.g., billions of CFUs per day). The purpose is to support gut barrier function and immune balance. Mechanistically, probiotics can adjust gut microbiota composition and influence immune cell behavior through gut-associated lymphoid tissue. In severely immunocompromised patients, there is a tiny risk of infection from some probiotic strains, so choices should be guided by a physician.
5. Zinc
Zinc is essential for immune cell development and function. Mild deficiency is common in chronic disease and poor nutrition. Typical supplemental doses are 10–25 mg/day of elemental zinc, limited in duration to avoid copper deficiency. Mechanistically, zinc supports normal function of T cells and other immune cells and helps maintain skin and mucosal barriers. Too much zinc can cause nausea and interfere with copper and iron metabolism.
6. Selenium
Selenium is a trace mineral involved in antioxidant enzymes such as glutathione peroxidase. Some evidence suggests that low selenium levels may impair immune response. Supplemental doses around 50–100 mcg/day are usual when deficiency is suspected. Mechanistically, selenium helps neutralize free radicals and supports thyroid and immune function. Excess selenium can cause hair loss, nail changes, and nerve problems, so it should not be taken in high doses without testing.
7. Vitamin C
Vitamin C is a water-soluble antioxidant and supports collagen production and immune defense. Typical oral doses range from 200–500 mg/day or more, but very high doses can cause diarrhea and kidney stones in susceptible individuals. Mechanistically, vitamin C supports white blood cell function and helps regenerate other antioxidants such as vitamin E. It may slightly shorten the duration of colds, but evidence in Castleman disease is indirect.
8. Green tea extract (EGCG)
Green tea contains catechins such as EGCG, which have antioxidant and mild anti-proliferative effects in lab studies. Supplemental doses vary; moderate intake is usually considered one to two cups of tea daily or standardized capsules under medical guidance. Mechanistically, EGCG can affect signaling pathways and free radicals, but clinical benefits in Castleman disease are not proven. High-dose extracts have been linked to liver injury in rare cases.
9. N-acetylcysteine (NAC)
NAC is a precursor to glutathione, one of the body’s main antioxidants. Oral NAC is used in several conditions to support liver health and mucus clearance. Typical supplements range from 600–1200 mg/day but must be supervised in complex patients. Mechanistically, NAC replenishes glutathione, helps neutralize oxidative stress, and may modulate inflammatory pathways. Side effects include nausea and, rarely, allergic reactions.
10. Coenzyme Q10 (CoQ10)
CoQ10 is involved in mitochondrial energy production and has antioxidant properties. It may be considered in patients with fatigue or those receiving certain cardiotoxic drugs, though evidence is limited. Typical doses range from 100–200 mg/day. Mechanistically, CoQ10 helps the cell’s “energy factories” (mitochondria) work and may reduce oxidative damage to heart and muscle cells. It can interact with blood thinners and should be discussed with the treating doctor.
Immune-supporting and regenerative drugs
Again, these are not standard treatments for Castleman disease itself, but may be used to support blood counts or recovery in special situations. Always specialist-guided.
1. Filgrastim (G-CSF)
Filgrastim is a granulocyte colony-stimulating factor that stimulates the bone marrow to produce more neutrophils. It is used when chemotherapy or the disease itself causes very low neutrophil counts. The purpose is to reduce infection risk and allow treatment to continue. Mechanistically, filgrastim binds to G-CSF receptors on precursor cells, promoting their growth and maturation.
2. Pegfilgrastim
Pegfilgrastim is a long-acting form of G-CSF, given less often (usually once per chemotherapy cycle). It has similar functions and mechanisms as filgrastim but stays longer in the body due to pegylation. The purpose is convenient, sustained neutrophil support. Side effects for both G-CSF drugs include bone pain and, rarely, spleen enlargement or rupture.
3. Epoetin alfa (Erythropoietin)
Epoetin alfa is a synthetic form of erythropoietin, stimulating red blood cell production. It is used in selected patients with significant anemia from chemotherapy or chronic disease when transfusions are frequent. The purpose is to improve hemoglobin and reduce transfusion reliance. Mechanistically, it acts on erythroid progenitor cells in the marrow. It can increase clot risk and blood pressure and must be carefully monitored.
4. Darbepoetin alfa
Darbepoetin is a longer-acting ESA. The mechanism and purpose are similar to epoetin alfa, but injections are less frequent, which can be more convenient. Dose and schedule depend on hemoglobin levels and treatment goals. As with all ESAs, using the lowest effective dose and following safety guidelines is important to reduce thrombotic complications.
5. Romiplostim
Romiplostim is a thrombopoietin receptor agonist given by injection to stimulate platelet production. It may be used in specific cases with severe thrombocytopenia (very low platelets) that limit therapy or cause bleeding. Mechanistically, it mimics thrombopoietin and stimulates megakaryocytes in the bone marrow. Side effects include headache, joint pain, and potential increased clot risk; bone marrow fibrosis is monitored with prolonged use.
6. Eltrombopag
Eltrombopag is an oral thrombopoietin receptor agonist. It has similar functions to romiplostim but is taken by mouth. The purpose is to support platelet counts, reduce bleeding, and allow cancer or immune therapy to proceed. Mechanistically, it promotes megakaryocyte proliferation and platelet production through TPO receptor activation. Side effects include liver enzyme changes, cataracts, and clot risk, so regular monitoring is essential.
Surgeries and procedures
1. Complete surgical excision of unicentric lymph node
For unicentric Castleman disease, complete surgical removal of the affected lymph node is the first-line and often curative treatment.Medscape+2BSH+2
The procedure may be done via open surgery or minimally invasive (laparoscopic or thoracoscopic) approaches, depending on location (chest, abdomen, neck). It is done to remove the disease focus and relieve compression on nearby structures. After successful excision, many patients need no additional therapy.
2. Debulking surgery
Sometimes the lymph node mass is very large or involves vital structures, and complete safe removal is not possible. In such cases, surgeons may perform debulking, removing as much disease tissue as they safely can. The purpose is to reduce symptoms (pain, compression of organs) and make subsequent treatments such as radiation or systemic therapy more effective. It is usually planned after multidisciplinary discussion.
3. Splenectomy
If the spleen is heavily involved with Castleman disease or greatly enlarged, a splenectomy (spleen removal) may be considered. The purpose is to reduce disease burden, improve blood counts, or relieve discomfort and early satiety from a large spleen. After splenectomy, patients have an increased lifelong risk of certain infections and need appropriate vaccines and sometimes prophylactic antibiotics.
4. Mediastinal or abdominal lymph-node dissection
In some patients, a more extensive lymph-node dissection in the chest (mediastinum) or abdomen is performed to remove multiple enlarged nodes in a region. The procedure is more complex and may involve thoracic or abdominal surgeons. The purpose is local disease control when many nodes in a single area are affected and other options are limited.
5. Central venous access device (port or PICC line) placement
Although not disease-curing, placement of a central venous catheter or port is an important procedure for patients receiving frequent IV treatments (siltuximab, rituximab, chemotherapy, blood products). It provides reliable venous access, reduces needle sticks, and makes infusions safer and more comfortable. The procedure is done under local anesthesia and imaging guidance. Risks include infection and thrombosis, so careful care of the line is critical.
Prevention strategies
You cannot always prevent Castleman disease, but you can reduce complications and improve outcomes:
Early evaluation of persistent swollen lymph nodes – see a doctor if a node stays enlarged for more than a few weeks.
Prompt treatment of infections and HIV – good control of HIV and other infections may lower risk and severity of HHV-8–associated MCD.
Regular follow-up with a hematologist experienced in Castleman disease, even when you feel well.
Vaccinations (flu, pneumococcal, COVID-19 and others as advised) to prevent serious infections.
Healthy lifestyle – balanced diet, regular activity, no smoking, limited alcohol to support immune and heart health.
Medication adherence – take treatments exactly as prescribed and attend all infusion appointments.
Monitoring for side effects – report fevers, new pain, neurological symptoms, or breathing problems early.
Avoiding unnecessary immunosuppressive drugs – always tell doctors you have Castleman disease before new medicines are started.
Cancer screening as recommended for age and risk, because lymphoma and other cancers are more common.
Psychological and social support, which helps maintain good self-care and adherence.
When to see a doctor urgently
You should seek immediate medical care (emergency department or urgent clinic) if you have Castleman disease and notice:
High fever, chills, or shaking
Trouble breathing, chest pain, or severe cough
Confusion, severe headache, or new neurological symptoms
Very fast heart rate, low blood pressure, or feeling like you might faint
Uncontrolled bleeding or many new bruises
You should contact your hematologist or treating doctor soon (within days) if you notice:
New or growing lymph node lumps
Night sweats, unintentional weight loss, or worsening fatigue
New abdominal swelling, early fullness after small meals, or pain
Persistent cough or mild fevers that do not go away
Any new symptom that worries you, especially after a change in treatment
Never stop a drug like siltuximab, rituximab, steroids, or chemotherapy on your own. Always discuss changes with your doctor.
What to eat and what to avoid
1. Eat: Colorful fruits and vegetables
Aim for many colors: leafy greens, berries, carrots, tomatoes, citrus. They provide vitamins, minerals, and antioxidants that help the immune system and protect cells from oxidative stress.
2. Eat: Lean proteins
Include fish, poultry, eggs, tofu, beans, and lentils. Protein supports muscle strength, wound healing, and blood cell production, which is important during systemic treatment and recovery.
3. Eat: Whole grains
Choose brown rice, oats, whole-wheat bread, and other whole grains. They give sustained energy and fiber, helping bowel function and blood sugar control.
4. Eat: Healthy fats
Use small amounts of olive oil, nuts, seeds, and avocado. These provide essential fatty acids and support heart and brain health. They also help absorb fat-soluble vitamins.
5. Eat: Probiotic and fermented foods (when safe)
Yogurt with live cultures, kefir, and some fermented foods may support gut microbiota and immunity. In severely immunocompromised patients, your doctor may advise on which foods are safe.
6. Avoid: Highly processed meats and deep-fried foods
Sausages, bacon, and very fried foods can promote inflammation and increase heart disease risk. They add unhealthy fats and salt without useful nutrients.
7. Avoid: Sugary drinks and excessive sweets
Soft drinks, energy drinks, and candy cause rapid blood sugar spikes and provide “empty calories,” which can worsen weight gain or metabolic problems and contribute to fatigue.
8. Avoid: Excessive salt
Too much salt from fast foods, packaged snacks, and instant soups can worsen high blood pressure and fluid retention, especially if steroids or some chemotherapy drugs are used.
9. Avoid: Alcohol, especially in large amounts
Alcohol stresses the liver, which already has to handle powerful medicines. It can also interact with many drugs. Your doctor may recommend avoiding alcohol completely during treatment.
10. Avoid: Raw or unpasteurized foods when very immunosuppressed
Raw eggs, raw seafood, unpasteurized dairy, and undercooked meats can carry germs that cause serious infection in patients with low immunity. Always follow food safety rules given by your care team.
Frequently asked questions
1. Is benign angiofollicular hyperplasia (Castleman disease) a cancer?
Castleman disease is not a classic cancer like lymphoma, but it is a lymphoproliferative disorder with abnormal growth of lymph node tissue and overproduction of inflammatory proteins. Some people with Castleman disease later develop lymphoma, so it is taken very seriously and monitored closely.Cancer.gov+2Wikipedia+2
2. What is the difference between unicentric and multicentric disease?
In unicentric disease, only one lymph node region is involved, and surgery can often cure it. In multicentric disease, many lymph node areas are affected, and the whole body shows inflammation with symptoms like fever, weight loss, and anemia. Multicentric disease usually needs long-term, systemic treatment.PMC+2BSH+2
3. Can Castleman disease be cured?
Many people with unicentric disease are cured with complete surgical excision. Multicentric disease is usually chronic. With modern biologic drugs such as siltuximab and rituximab and well-designed guidelines, many patients can reach long-lasting remission, but ongoing follow-up is needed.PMC+1
4. What causes benign angiofollicular hyperplasia?
The exact cause is not fully known. Some cases are linked to HHV-8 infection and often occur in people with HIV. Others are idiopathic, meaning no clear trigger is found. Researchers know that IL-6 and other cytokines play a central role in the abnormal immune activation and lymph node changes.Wikipedia+2PMC+2
5. Is it contagious?
Castleman disease itself is not contagious. However, viruses associated with some forms (like HHV-8 and HIV) can be transmitted between people. Preventing those infections through safe practices and appropriate treatment is important.
6. How is Castleman disease diagnosed?
Diagnosis usually requires excisional lymph-node biopsy, where a whole node is removed and examined under a microscope. Doctors also use blood tests, CT or PET scans, and sometimes bone marrow biopsy to assess the extent of disease and rule out lymphoma and other conditions.PMC+2ScienceDirect+2
7. Why is IL-6 so important in this disease?
IL-6 is a cytokine that stimulates inflammation, fever, and production of acute phase proteins like CRP and fibrinogen. In many Castleman patients, IL-6 is overproduced, leading to fever, anemia, fatigue, and elevated inflammatory markers. Blocking IL-6 with drugs like siltuximab or tocilizumab helps relieve these symptoms.FDA Access Data+2FDA Access Data+2
8. How often will I need follow-up?
Follow-up frequency depends on disease type, activity, and treatment. After surgery for unicentric disease, visits may be every 6–12 months initially. In multicentric disease on active therapy, visits are usually more frequent (every few weeks to months) for physical exams, blood tests, and sometimes imaging. Your team will create a personalized schedule.
9. Can I live a normal life with Castleman disease?
Many patients, especially with well-controlled disease, can work, study, and enjoy family life, although adjustments may be needed for fatigue and medical appointments. Good communication with your care team, employers, and family helps you plan around treatments and energy levels.
10. Does diet really make a difference?
Diet cannot cure Castleman disease, but good nutrition supports the immune system, helps maintain strength, and improves tolerance to surgery and medicines. Malnutrition or very unhealthy diets can make treatment harder and recovery slower. A dietitian can help build a realistic, enjoyable plan.
11. Are there clinical trials for Castleman disease?
Yes. Because it is rare, many advanced treatments and new drugs are tested in clinical trials run at specialized centers. If your disease is difficult to control or if you are interested in research participation, your hematologist can help you find appropriate trials through clinical trial registries and Castleman disease networks.PMC+2DrugBank+2
12. Can pregnancy be safe with Castleman disease?
Some women with stable disease have successful pregnancies, but it requires careful planning with hematology, obstetrics, and sometimes high-risk pregnancy specialists. Some drugs used for Castleman disease are harmful to the fetus (e.g., thalidomide, lenalidomide, many chemotherapies). Effective contraception is essential during such treatments, and any pregnancy plan should be discussed well in advance.
13. What about children with Castleman disease?
Castleman disease can rarely affect children. Management should be in a pediatric hematology/oncology center with experience in lymphoproliferative disorders. Treatment choices and doses are adjusted for age and growth, and long-term follow-up is important to monitor development, school performance, and late treatment effects.
14. How important is mental health care?
Mental health care is very important. Anxiety, depression, and fear of relapse are common. Early involvement of psychologists or psychiatrists, support groups, and open conversations with family can greatly improve quality of life and treatment adherence. There is no weakness in asking for help; it is part of good medical care.
15. Where can I find reliable information?
Reliable sources include national hematology societies, rare disease databases, and dedicated Castleman disease research groups and foundations. These organizations provide patient-friendly explanations, guideline summaries, and links to expert centers and clinical trials.CDCN+3GARD Information Center+3PMC+3
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.
