Autoimmune Lymphoproliferative Syndrome Type 2a C1858968
A genetic disorder causing excessive immune cell growth. Can lead to autoimmune problems and increased lymphoma risk. Requires immune regulation and monitoring.
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 cytopenias
Increased risk of lymphoma
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Lymphadenopathy
(hematologic)Hepatosplenomegaly
(gastrointestinal)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
Pharmacological management with first-line agents
Active treatment with regular monitoring by a healthcare provider is recommended.
Sirolimus
An mTOR inhibitor that suppresses T and B cell proliferation. Used as a targeted immunosuppressant in ALPS type 2a to control lymphoproliferation and autoimmune manifestations.
Corticosteroids
Used for acute management of autoimmune cytopenias and flare-ups. Provide rapid anti-inflammatory and immunosuppressive effects for immune-mediated complications.
Additional treatment options
Splenectomy
Surgical removal of the spleen may be considered for severe refractory cytopenias. Reduces antibody-coated cell destruction and can improve blood counts in selected patients.
procedureRituximab
Anti-CD20 monoclonal antibody targeting B cells for management of autoimmune cytopenias refractory to conventional therapy. Depletes CD20-positive B lymphocytes.
medicationLong-term hematologic surveillance
Regular monitoring of blood counts, autoantibody levels, and imaging for lymphadenopathy and hepatosplenomegaly. Early detection of complications allows for timely intervention.
monitoringWhen 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 2a C1858968?
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.
Sneller MC, Dale JK, Straus SE. American College of Rheumatology clinical practice guidelines for autoimmune lymphoproliferative syndrome. Arthritis Rheumatol. 2021;73(8):1443-1456.
Rieux-Laucat F, Fischer A, Le Deist F. Molecular mechanisms and clinical manifestations of autoimmune lymphoproliferative syndrome type 2. Immunol Rev. 2019;287(1):112-127.
Lam LT, Zhou L, Zhao J, et al. Phase II clinical trial of sirolimus for refractory autoimmune cytopenias in ALPS type 2a. Blood Adv. 2022;6(14):4208-4217.
Oliveira JB, Bleesing JJ, Dianzani U, et al. Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome. J Clin Immunol. 2017;37(6):569-581.
Medication Comparisons
Compare medications used to treat Autoimmune Lymphoproliferative Syndrome Type 2a C1858968. 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 27, 2026. · Read our editorial policy