CASP8 Autoimmune Lymphoproliferative Syndrome (CASP8-ALPS / Caspase-8–Related ALPS-like Disease)

Autoimmune lymphoproliferative syndrome (CASP8-ALPS / caspase-8–related ALPS-like disease) is a very rare genetic immune disorder in which a change in the CASP8 gene disrupts a key “self-destruct” signal (apoptosis) that normally keeps immune cells in balance. When this pathway fails, white blood cells can build up in lymph nodes and the spleen (lymphoproliferation) and the immune system may misfire, sometimes causing low blood counts (autoimmune cytopenias) or repeated infections. Doctors often call this picture ALPS-like because it resembles classical Autoimmune Lymphoproliferative Syndrome, which is usually caused by changes in the FAS gene; CASP8 sits in the same pathway. Not all people with caspase-8 deficiency develop full ALPS; some mainly have immunodeficiency with swollen lymph nodes/spleen and frequent infections. SpringerLink+3NCBI+3PMC+3

Classic ALPS most often involves FAS (CD95) and features “double-negative T cells” (DNT: TCRαβ+, CD4–, CD8–) and autoimmune cytopenias; CASP8 deficiency can show a similar build-up of lymphocytes and DNT cells, but earlier reports emphasized infections more than autoimmunity, and newer case descriptions show broader immune dysregulation. In practice, many clinicians group CASP8 defects among ALPS/ALPS-like disorders in the FAS pathway. NCBI+2ASH Publications+2

CASP8 autoimmune lymphoproliferative syndrome is a rare, inherited immune system disease in which a key protein called caspase-8 does not work properly. Caspase-8 normally helps immune cells (like T cells and B cells) “switch off” at the right time by a safe self-destruct step (apoptosis), and it also helps them activate correctly when needed. When CASP8 is faulty, immune cells can both accumulate abnormally (causing big lymph nodes and spleen) and misfire (causing autoimmune attacks on your own blood cells), while at the same time being too weak against infections, especially some herpes viruses. Some people also develop gut inflammation similar to inflammatory bowel disease and, less commonly, granulomas (tiny clusters of immune cells) in different organs. Doctors think of it as an ALPS-like condition: it shares many features with classic ALPS, but CASP8 patients often have more infection-prone immunodeficiency mixed with the autoimmunity and lymphoproliferation. Gastrojournal+3NCBI+3Orpha+3

Other names

• Autoimmune lymphoproliferative syndrome – recurrent viral infections due to CASP8 deficiency (Orphanet label; sometimes shortened to ALPS with recurrent viral infections due to CASP8) Orpha

• Caspase-8 deficiency state (CEDS) or CASP8 deficiency (often used in medical papers; described as ALPS-like with immunodeficiency) PMC+1

• ALPS-like disease due to CASP8 variants (research descriptions highlighting overlap with classic ALPS) Haematologica

Types

Because this is rare, there isn’t a single official “type list.” Doctors usually group patients by how the disease behaves and by the kind of CASP8 mutation. These practical types help plan tests and care:

1. Immunodeficiency-predominant type. Recurrent viral (e.g., HSV/EBV), sinus, or chest infections stand out; lymph nodes/spleen may be present but not the main problem. Orpha+1

2. Autoimmunity-predominant (ALPS-like) type. Big lymph nodes and spleen plus autoimmune cytopenias (low platelets, low red cells, low neutrophils) dominate; infections occur but are secondary. NCBI

3. Entero-inflammatory type. Early or chronic IBD-like colitis with weight loss, pain, diarrhea, sometimes with granulomas in organs. Haematologica+1

4. Adult-onset granulomatous infiltration type. Later presentation with multi-organ lymphocytic infiltrates and granulomas (lung, liver, marrow, CNS), sometimes severe. PMC

5. Genotype-based types.
   • Biallelic (homozygous/compound heterozygous) loss-of-function variants: usually earlier, more severe disease.
   • Hypomorphic or dominant-negative variants: sometimes milder or later onset, ALPS-like. (Patterns summarized across case series/reviews.) Haematologica+1

---

Causes

Here “cause” means a driver or contributor that leads to the disease features (the root genetic problem plus the downstream immune pathway problems and common clinical triggers).

1. Inherited CASP8 gene variants (loss-of-function or hypomorphic): the core cause; the protein cannot execute normal cell-death signaling. BioMed Central

2. Defective Fas/FasL pathway signaling: Fas-mediated apoptosis is blunted; autoreactive lymphocytes persist. (Classic ALPS pathway, also relevant here.) NCBI

3. Impaired T-cell activation: T cells do not activate or proliferate properly, weakening defense against infections. BioMed Central

4. Reduced IL-2 production/signaling: contributes to poor T-cell growth and regulation. BioMed Central

5. Abnormal B-cell help and antibody responses: leading to weak responses to vaccines and some infections. malacards.org

6. Faulty NK-cell activation: impaired killing of infected or abnormal cells. malacards.org

7. Excess survival of autoreactive lymphocytes: increases risk of autoimmune cytopenias. NCBI

8. Necroptosis/inflammation imbalance: caspase-8 normally restrains RIPK pathways; loss can tilt toward inflammatory cell death and tissue damage. (Shown in models; supports human biology.) Nature

9. Herpesvirus triggers (HSV/EBV/VZV): common, sometimes severe infections in CASP8 deficiency can flare disease activity. Orpha+1

10. Chronic mucosal injury in the gut: dysregulated responses to microbes/food antigens promote IBD-like inflammation. Gastrojournal

11. Cytokine imbalance (e.g., dysregulated pro-/anti-inflammatory signals) that caspase-8 helps tune. ScienceDirect

12. Environmental infections besides herpesviruses (e.g., respiratory pathogens) that exploit the immunodeficiency. malacards.org

13. Vaccine response defects: poor serologic responses can leave gaps in protection and allow repeated infections. malacards.org

14. Secondary immune exhaustion from ongoing activation trying to control infections. (Mechanistic inference supported by functional studies.) BioMed Central

15. Granuloma formation in tissues, reflecting chronic, dysregulated T-cell responses to persistent antigens. PMC

16. Overlap with classic ALPS biomarkers/pathways: similar downstream effects (lymphadenopathy/splenomegaly, cytopenias). NCBI

17. Genetic background/modifiers that change severity or age at onset (general ALPS insight extrapolated to CASP8). NCBI

18. Autoantibody formation against blood cells because self-tolerance is not enforced well. (ALPS principle applied to CASP8.) NCBI

19. Defective dendritic cell apoptosis/education (applies in ALPS family; can amplify autoimmunity). NCBI

20. Non-apoptotic signaling defects (caspase-8 also fine-tunes inflammatory gene programs; loss skews them). ScienceDirect

---

Symptoms and signs

1. Painless, chronic swollen lymph nodes (neck, underarms, groin). Orpha

2. Enlarged spleen ± liver, sometimes causing fullness or early satiety. Orpha

3. Frequent viral infections, especially herpes family (cold sores, shingles, EBV-like illness). Orpha

4. Recurrent sinus/chest infections (cough, wheeze, pneumonia). malacards.org

5. Easy bruising, nosebleeds, or petechiae from low platelets (autoimmune thrombocytopenia). NCBI

6. Pale skin, fatigue, shortness of breath from autoimmune hemolytic anemia or other cytopenias. NCBI

7. Fevers and malaise during flares or infections. Haematologica

8. Abdominal pain, diarrhea, weight loss suggesting IBD-like gut inflammation. Gastrojournal

9. Mouth ulcers or sore throat during immune flares/infections (common but non-specific). Haematologica

10. Skin rashes (autoimmune or infection-related). Haematologica

11. Night sweats or persistent fatigue with active lymphoproliferation. NCBI

12. Enlarged tonsils/adenoids due to lymphoid overgrowth. NCBI

13. Granuloma-related organ symptoms (e.g., cough/breathlessness if lungs involved). PMC

14. Poor vaccine responses noticed as infections despite immunization. malacards.org

15. Growth or school performance issues from chronic illness and inflammation (general ALPS/IBD overlap). Gastrojournal

---

Diagnostic tests

A) Physical examination

1. Comprehensive lymph node exam. The doctor checks size, texture, and tenderness of many nodal areas; chronic, non-tender nodes suggest lymphoproliferation rather than acute infection. (Core ALPS feature.) NCBI

2. Abdominal palpation for spleen and liver. A firm, enlarged spleen is common; size guides urgency and bleeding risk discussions. NCBI

3. Skin and mucosa check. Looks for bruises/petechiae (thrombocytopenia), pallor (anemia), ulcers/rashes (autoimmunity/infection). NCBI

4. Respiratory exam. Wheeze or crackles can point toward recurrent chest infections or granulomatous lung disease. PMC

5. Growth and nutrition review. Weight/height percentiles and signs of malnutrition can point to gut involvement. Gastrojournal

B) “Manual”/bedside clinical tests

6. Fever diary and symptom timeline. A structured record helps separate infection-driven flares from autoimmune flares.

7. Spleen percussion (“splenic dullness”). A quick bedside method to support splenomegaly between imaging checks.

8. Stool symptom score (with fecal sampling plan). Guides whether to move to lab markers and colonoscopy for IBD-like disease. Gastrojournal

9. Medication/immunization history review. Identifies poor vaccine responses and excludes drug-induced cytopenias. malacards.org

10. Family pedigree. Looks for consanguinity or relatives with similar problems, supporting a recessive pattern. PMC

C) Laboratory & pathological tests

11. Complete blood count (CBC) with smear. Detects anemia, leukopenia/neutropenia, thrombocytopenia; smear looks for hemolysis clues. (Typical in ALPS-like disease.) NCBI

12. Direct antiglobulin (Coombs) test. Confirms autoimmune hemolysis if positive. NCBI

13. Immunoglobulin levels and vaccine titers. Low or non-protective titers signal impaired humoral immunity. malacards.org

14. Lymphocyte subsets by flow cytometry. Quantifies T, B, NK cells; may show abnormal activation or numbers; in classic ALPS, double-negative T cells can be high (CASP8 cases can vary). NCBI

15. Functional apoptosis assay (Fas-induced apoptosis). Measures whether lymphocytes die appropriately when Fas is triggered; defective in ALPS pathway disorders and in some CASP8 patients. NCBI

16. Inflammatory markers (CRP/ESR) and fecal calprotectin. Support IBD-like activity assessment. Gastrojournal

17. Autoimmune panels (DAT, anti-platelet antibodies, ANA if clinically needed). Help document autoimmunity drivers of cytopenias. NCBI

18. Tissue pathology when indicated (e.g., lymph node, gut, lung). Can show granulomas or chronic lymphoid infiltration; excludes malignancy. PMC

D) Functional/“electrodiagnostic-style” immune tests

19. T-cell proliferation/activation assays (mitogen/antigen induced). CASP8 deficiency can blunt T-cell activation and proliferation, which these assays reveal. BioMed Central

20. NK-cell cytotoxicity/activation assays. May uncover impaired NK function that contributes to infections. malacards.org

E) Imaging tests

21. Abdominal ultrasound. Safe way to track spleen and liver size over time. NCBI

22. Chest CT (or high-resolution CT) when needed. Evaluates recurrent infections or granulomatous lung disease. PMC

23. FDG-PET/CT in selected cases. Helps map active lymphoproliferation when nodes are widespread or to exclude lymphoma if the picture is unclear (borrowed from ALPS practice). NCBI

24. Endoscopy/colonoscopy with biopsies for GI symptoms. Defines IBD-like inflammation and guides therapy. Gastrojournal

25. MRI for organ-specific concerns (e.g., CNS involvement). Non-invasive way to assess granulomatous or inflammatory lesions. PMC

Non-pharmacological treatments (therapies & other supports)

1. Education & safety plan: Learn infection warning signs and when to seek urgent care; keep care summaries handy. This reduces delay for serious infections. PMC

2. Vaccination optimization: Keep routine inactivated vaccines current; plan timing around immunosuppressants; avoid live vaccines during high-dose immune suppression. CDC+2PMC+2

3. Antibiotic fever protocol after splenectomy (if ever done): Immediate medical evaluation and prompt antibiotics with any fever; daily prophylaxis often advised. Splenectomy is generally avoided in ALPS because of lifelong sepsis risk. PMC+1

4. Infection prevention habits: Hand hygiene, prompt dental care, safe food/water practices; these lower exposure in immunocompromised patients. CDC

5. Nutritional support: Balanced, protein-adequate diet and micronutrients to support recovery from infections and cytopenias; tailor to appetite and GI tolerance. (Supportive care guidance from pediatric ALPS resources.) GOSH Hospital site

6. Activity pacing: Gentle, regular activity with rest to counter fatigue and deconditioning during flares. GOSH Hospital site

7. Spleen protection precautions: Avoid contact sports with splenomegaly to reduce rupture risk; use seatbelts properly. PMC

8. Psychosocial support: Counseling and peer support reduce anxiety from chronic lymphadenopathy and repeated medical visits. GOSH Hospital site

9. School/work plans: Flexible attendance during infections, vaccination scheduling, and medication monitoring. GOSH Hospital site

10. Sun/skin care and oral hygiene: Reduces secondary skin/oral infections when neutropenia occurs. CDC

11. Household vaccination strategy: Encourage family to get influenza and other recommended inactivated vaccines; reduces exposure. CDC

12. Travel preparation: Pre-travel vaccines (inactivated), malaria prophylaxis if needed, and medical letters for medications. CDC

13. Fever action card: Clear thresholds (e.g., ≥38.0°C), emergency contacts, nearest hospital. PMC

14. Bone health basics if corticosteroids are needed (calcium/vitamin D diet, weight-bearing activity). PMC

15. Medication calendar & labs tracking: Improves adherence and early detection of low immunoglobulins on rituximab or cytopenias on other agents. AAAAI

16. Home infection kit (as directed): Thermometer, oral rehydration, masks; never replace medical assessment. CDC

17. Allergy/antibiotic card post-splenectomy: Shows need for rapid antibiotics if fever; prevents delays. PMC

18. Avoid unnecessary biopsies/surgeries: Lymph node enlargement is often benign in ALPS-like disorders; surgery rarely helps and carries risks. Medscape

19. Care coordination: Immunology, hematology, primary care, and (if needed) infectious diseases work together. GOSH Hospital site

20. Clinical trial awareness: Ask about trials of targeted therapies in inborn errors of immunity. SpringerLink

---

Drug treatments

1. Sirolimus (rapamycin; mTOR inhibitor): Often the most effective steroid-sparing agent in ALPS/ALPS-like disease. It can shrink lymph nodes/spleen, reduce DNT cells, and control autoimmune cytopenias. Typical pediatric starts ~1–2 mg/m²/day (or weight-based) with drug-level monitoring; adults often 1–2 mg/day titrated. Side effects can include mouth sores, high lipids, and infection risk. Evidence includes case series and trials showing high response rates and durable control. PMC+2PMC+2

2. Mycophenolate mofetil (MMF; antimetabolite): Useful for autoimmune cytopenias and as a steroid-sparing drug; responses in ~65–90% across pediatric reports. It may not consistently shrink lymphoproliferation. Pediatric dosing often ~600 mg/m² twice daily; adults commonly 1 g twice daily, adjusted for tolerance. GI upset and cytopenias can occur; monitor counts. PubMed+2PMC+2

3. Short course corticosteroids (e.g., prednisone): Effective for acute flares of autoimmune hemolysis/thrombocytopenia but not for long-term control due to toxicity (bone loss, diabetes, infections). Doses vary by flare severity (e.g., 1–2 mg/kg/day initially, then taper). Aim to move to steroid-sparing therapy. PMC

4. IVIG (intravenous immunoglobulin): Helps immune thrombocytopenia/hemolysis crises and replaces IgG if hypogammaglobulinemia or serious infections occur (especially after rituximab). Typical dosing for ITP crisis 1–2 g/kg; for replacement 0.4–0.6 g/kg every 3–4 weeks. Headache and aseptic meningitis are known risks. AAAAI

5. Rituximab (anti-CD20): Can control severe autoimmune cytopenias when steroids/IVIG fail, but it raises infection risk by lowering B cells and can cause prolonged hypogammaglobulinemia, especially in children; vaccine responses are blunted. If used, plan vaccinations and Ig monitoring. Typical dose 375 mg/m² weekly × 4 or equivalent. PMC+2JAMA Network+2

6. Azathioprine: Sometimes used as an adjunct for cytopenias when MMF/sirolimus are not suitable. Dose ~1–2 mg/kg/day with TPMT testing to reduce myelotoxicity; monitor blood counts and liver enzymes. Evidence is less robust than MMF/sirolimus in ALPS. Medscape

7. Cyclosporine/tacrolimus (calcineurin inhibitors): May help certain autoimmune manifestations; require drug-level monitoring and blood-pressure/kidney checks. Used when first-line options are unsuitable. Medscape

8. Thrombopoietin receptor agonists (eltrombopag/romiplostim): For refractory immune thrombocytopenia to raise platelets and avoid splenectomy; monitor liver tests and thrombotic risk. ASH Publications

9. TMP-SMX or penicillin prophylaxis (selected patients): After splenectomy (ideally avoided) or with severe hypogammaglobulinemia to reduce severe bacterial infections; follow local guidelines. PMC

10. Antiviral prophylaxis (individualized): Considered in profound B-cell depletion or frequent herpesvirus reactivations; specialist-directed. malacards.org

11. Sirolimus monotherapy (long-term): Specific mention because evidence supports durable control with acceptable safety when monitored. Lipids, mouth ulcers, and renal function require checks. ScienceDirect+1

12. Pulse methylprednisolone for life-threatening flares: Short, high-dose IV steroid pulses can rapidly control hemolysis; bridge to safer chronic therapy. PMC

13. Rituximab + IVIG support: If rituximab is chosen, plan IgG monitoring and replacement to prevent recurrent infections. AAAAI

14. Vaccination scheduling around drugs: Not a drug per se, but critical medication-timing guidance (e.g., hold rituximab for non-live vaccines when possible; avoid live vaccines on high-dose immunosuppression). PMC+1

15. Growth-factor support (G-CSF): For severe neutropenia with infections while other therapies take effect; use judiciously under hematology guidance. (Supportive hematology practice.) PMC

16. Second-line antimetabolites (e.g., methotrexate in select scenarios): Far less commonly used; monitor liver and blood counts; limited ALPS-specific data. PMC

17. Plasmapheresis (rare rescue): For fulminant autoimmune hemolysis unresponsive to drugs, as a temporary bridge. (General autoimmune cytopenia practice.) PMC

18. Cyclophosphamide (selected refractory cases): Powerful immunosuppression; infection, infertility, and malignancy risks must be weighed heavily; generally avoided if safer options work. PMC

19. Sirolimus + MMF (specialist-directed combination): Sometimes combined for difficult disease; monitor closely for overlapping cytopenias/infections. PMC

20. Hematopoietic stem cell transplantation (HSCT): Curative potential for severe, refractory disease with life-threatening complications; carries transplant risks and needs expert centers. Medscape

---

Dietary molecular supplements

There are no supplements proven to treat CASP8-ALPS itself. Any use should focus on general health and be cleared with your clinician (drug interactions and infection risk matter). The items below reflect supportive roles; they do not replace medical therapy.

1. Vitamin D (correct deficiency; immune-modulatory effects; typical 600–2000 IU/day individualized). Monitor levels to avoid toxicity. CDC

2. Calcium (bone support if on steroids; dose per age/diet; avoid excess). PMC

3. Folic acid/B-vitamins (replace deficiencies that worsen anemia; dosing individualized). PMC

4. Oral iron (if iron-deficiency anemia is documented; avoid if hemolysis without iron deficiency). PMC

5. Zinc (correct deficiency that impairs immunity; excessive zinc harms copper balance). CDC

6. Omega-3 fatty acids (heart/lipid support if sirolimus raises triglycerides; dose ~1–2 g/day EPA+DHA after clinician approval). ScienceDirect

7. Probiotics (avoid in severely immunocompromised or central lines; limited evidence; discuss first). CDC

8. Multivitamin (basic coverage when appetite is poor; avoid megadoses). CDC

9. Oral rehydration salts (during febrile illnesses to maintain hydration). CDC

10. Protein-rich nutrition shakes (as a bridge during illness-related anorexia; check sugar content and tolerance). GOSH Hospital site

---

Immunity-booster / regenerative / stem-cell” drugs

In CASP8-ALPS, “boosting immunity” is not about over-stimulating the immune system; it’s about restoring balance—preventing destructive autoimmunity and protecting against infection.

1. IVIG (immune replacement when IgG is low): Replaces missing antibodies; reduces serious infections. Dose 0.4–0.6 g/kg every 3–4 weeks (individualized). AAAAI

2. Sirolimus (immune re-balancer): Targets overactive lymphocytes and often normalizes DNT cells; lowers autoimmune flares without broad steroid toxicity. Dosed/titrated with drug levels. PMC

3. MMF (autoimmunity-tamer): Reduces T/B-cell proliferation to quiet cytopenias; monitor for infection/cytopenias. Doses as above. PubMed

4. Vaccinations (immune education): “Trains” immunity safely with inactivated vaccines; schedule around B-cell–depleting drugs and avoid live vaccines under high-dose immunosuppression. PMC+1

5. Antimicrobial prophylaxis (functional immune aid): TMP-SMX or penicillin (post-splenectomy or recurrent infections) lowers life-threatening infection risk. PMC

6. HSCT (regenerative/cellular cure for the immune system): Replaces the faulty hemato-immune system when disease is severe and unresponsive; high-risk, specialist-only. Medscape

---

Surgeries (and why)

1. Lymph node biopsy (selective): Only if doctors suspect lymphoma or another diagnosis; many ALPS-like nodes are benign and don’t need removal. Medscape

2. Splenectomy (generally avoided): Historically used for refractory cytopenias, but strongly discouraged because of lifelong sepsis risk and limited long-term benefit. PMC+1

3. Central line placement (if necessary): For difficult IV access in patients needing frequent infusions; infection-prevention measures are critical. CDC

4. HSCT (see above): A transplant, not conventional surgery; considered for severe, refractory disease in expert centers. Medscape

5. Emergency splenic care: If traumatic splenic rupture risk is high due to massive splenomegaly, surgical teams may be involved; prevention is better than surgery. PMC

---

Preventions

• Stay vaccine-current with inactivated vaccines; time them around drugs like rituximab. PMC

• Avoid live vaccines while on high-dose immunosuppression or soon after rituximab. PMC

• Seek early care for fever, especially after splenectomy or on B-cell–depleting therapy. PMC

• Hand hygiene and masks during outbreaks or travel. CDC

• Dental and skin care to reduce infection portals. CDC

• Medication adherence & lab monitoring (drug levels, counts, IgG). ScienceDirect

• Avoid contact sports with splenomegaly. PMC

• Healthy sleep and nutrition to support recovery from infections. GOSH Hospital site

• Clinic coordination (immunology/hematology/ID) for unified plans. GOSH Hospital site

• Carry a medical summary (diagnosis, meds, allergy, “asplenia protocol” if relevant). PMC

---

When to see a doctor

Call or go to urgent care now for fever ≥38.0 °C, shaking chills, breathing trouble, severe abdominal pain (risk to big spleen), uncontrolled bleeding/bruising, extreme fatigue/pallor, or confusion. These may signal serious infection, severe anemia/platelets, or splenic issues. Immunocompromised people should not “wait it out.” PMC

---

What to eat / what to avoid

• Eat a balanced, protein-adequate diet; include iron-rich foods only if iron is low. PMC

• Eat fruit/veg that are well washed; safe food handling reduces infections. CDC

• Eat calcium + vitamin D (diet and supplements as advised) if using steroids. PMC

• Drink enough fluids, especially during fevers. CDC

• Limit ultra-processed sugar/fats if sirolimus raises lipids; consider omega-3s with clinician approval. ScienceDirect

• Avoid raw eggs/meats/unpasteurized products (foodborne infection risk). CDC

• Avoid alcohol excess (worsens cytopenias and drug side effects). PMC

• Avoid herbal megadoses or “immune boosters” without clearance (drug interactions, infection risks). CDC

• Time supplements/meds per your clinic (e.g., separate MMF from certain foods to reduce GI upset). PubMed

• Review any new diet/supplement with your specialist before starting. CDC

---

FAQs

1) Is CASP8-ALPS the same as classic ALPS?
No. They share the same apoptosis pathway, and symptoms can overlap. CASP8 disease has more infection-prone immunodeficiency in many reports and variable autoimmunity. NCBI

2) How is it diagnosed?
Through clinical features (big nodes/spleen, cytopenias), DNT cell measurement, apoptosis function tests, and genetic testing for CASP8/FAS-pathway genes. ASH Publications+1

3) Is it curable?
Medicines can control it well. HSCT may cure severe, refractory cases but has important risks. Medscape

4) What treatment works best?
Sirolimus is frequently effective and better tolerated long-term than steroids; MMF helps cytopenias. ScienceDirect+1

5) Are steroids safe long-term?
They work quickly but have many side effects; doctors try to taper and switch to steroid-sparing drugs. PMC

6) Should the spleen be removed?
Generally no; splenectomy raises lifelong sepsis risk in ALPS/ALPS-like disease and is avoided if at all possible. PMC+1

7) Are vaccinations safe?
Inactivated vaccines are recommended; time them around drugs like rituximab. Live vaccines are avoided during significant immunosuppression. CDC+1

8) What about rituximab?
It can help stubborn cytopenias but may cause prolonged low antibodies and reduced vaccine responses; monitoring and IVIG support may be needed. AAAAI+1

9) Does this increase cancer risk?
Classical ALPS carries an increased lymphoma risk; careful follow-up is standard. CASP8 data are limited but pathway awareness applies. PMC

10) Can I exercise?
Yes, but avoid contact sports if your spleen is enlarged. PMC

11) Diet changes to cure it?
No diet cures CASP8-ALPS. Nutrition supports overall health; medicines treat the immune imbalance. GOSH Hospital site

12) How often are labs needed?
Regular counts, immunoglobulins (especially with rituximab), and drug levels for sirolimus; schedule is individualized. ScienceDirect+1

13) Can children outgrow it?
Lymphadenopathy sometimes softens with age in ALPS, but genetic defects persist; ongoing follow-up is required. PubMed

14) Are clinical trials available?
Sometimes; ask your center about trials for inborn errors of immunity/ALPS-like disease. SpringerLink

15) Who should coordinate my care?
An immunologist/hematologist with experience in primary immune regulatory disorders (PIRDs), plus infectious diseases and primary care. GOSH Hospital site

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: September 29, 2025.

PDF Documents For This Disease Condition References