Autoimmune Lymphoproliferative Syndrome Unknown Underlying Mutation C1519711
A rare disorder where immune cells don't die normally, causing enlarged lymph nodes, spleen problems, and autoimmune symptoms. Can affect children and adults. Treated with medications to suppress the overactive immune response.
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 thrombocytopenia
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Lymphoproliferation
(immunologic)Chronic lymphadenopathy
(hematologic)Defective Fas-mediated apoptosis
(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
Empiric immunosuppression
Since the underlying genetic mutation is unknown, broad-spectrum immunosuppression is used to control symptoms while awaiting genetic clarification.
Targeted drug therapy with dose adjustment
Active treatment with regular monitoring by a healthcare provider is recommended.
Genetic testing and counseling
Ongoing evaluation for identifiable mutations guides prognosis and helps family members understand inheritance patterns.
Additional treatment options
Monoclonal antibody therapy
Rituximab or similar agents targeting B-cells may be employed for autoimmune cytopenias refractory to conventional therapy.
medicationNutritional support for cytopenias
Iron, folate, and vitamin B12 supplementation supports red blood cell production when deficiencies are identified through testing.
dietaryActivity modification during cytopenias
Avoid contact sports and high-risk activities when platelet counts are low to prevent bleeding complications.
lifestyleWhen 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 Unknown Underlying Mutation C1519711?
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.
Oliveira JB, Bleesing JJ, Dianzani U, et al. International Consensus Conference on ALPS: Diagnostic criteria and classification. J Clin Immunol. 2022.
Rao VK, Straus SE. Autoimmune lymphoproliferative syndrome: Advances in diagnosis and treatment. Immunol Allergy Clin North Am. 2020.
Bracaglia C, Caiello I, De Luca G, et al. Efficacy of sirolimus in patients with autoimmune lymphoproliferative syndrome. Blood. 2023.
Price S, Shaw PA, Seitz A, et al. Influence of germline FAS mutations on lymphocyte apoptosis and serum cytokine levels in ALPS. Clin Immunol. 2021.
Medication Comparisons
Compare medications used to treat Autoimmune Lymphoproliferative Syndrome Unknown Underlying Mutation C1519711. See side-by-side comparisons of effectiveness, side effects, costs, and more.
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 20, 2026. · Read our editorial policy