Autoimmune Lymphoproliferative Syndrome Type 4 C2674723
A subtype of this lymphoproliferative syndrome with distinct genetic features. Treatment involves immunosuppression and regular surveillance.
Key facts at a glance
- ICD-10
- D89.9
- ICD-11
- 3A00.0
- Body system
- immunologic
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Autoimmune hemolytic anemia
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Lymphadenopathy
(hematologic)Splenomegaly
(immunologic)Elevated double negative T cells
(immunologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Immunomodulatory therapy
B-cell targeted therapies and cytokine modulators help control the autoimmune lymphoproliferation characteristic of ALPS type 4. Treatment response varies by genetic subtype.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Comprehensive hematologic monitoring
Regular complete blood counts and flow cytometry assessment track disease activity and detect hematologic complications early.
Additional treatment options
Corticosteroid therapy
Systemic corticosteroids provide anti-inflammatory and immunosuppressive effects for acute flares. Long-term use requires bone density monitoring.
medicationPsychosocial support and counseling
Chronic illness counseling helps patients and families cope with the psychological burden of a lifelong genetic condition requiring ongoing treatment.
psychologicalStress reduction techniques
Mindfulness, meditation, and relaxation therapies support immune regulation and improve quality of life as adjunctive therapy.
complementaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Autoimmune Lymphoproliferative Syndrome Type 4 C2674723?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Leiding JW, Okada S, Hagin D, et al. Autoimmune Lymphoproliferative Syndrome: Diagnosis and Management. J Clin Immunol. 2018;38(6):673-698.
Ferrero G, Moshous D, Cirillo E, et al. Clinical Practice Guidelines for Autoimmune Lymphoproliferative Syndrome. Ital J Pediatr. 2020;46(1):123-134.
Atrash S, Dalal J, Sheikh F, et al. Sirolimus for the Treatment of Autoimmune Lymphoproliferative Syndrome. Pediatr Blood Cancer. 2021;68(4):e28942.
Shah S, Wu E, Rao VK, Lenardo MJ. Novel FASLG Mutation in Autoimmune Lymphoproliferative Syndrome Type 4. J Clin Immunol. 2019;39(2):142-158.
Medication Comparisons
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View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 27, 2026. · Read our editorial policy