Autoimmune Lymphoproliferative Syndrome Type 2b C1846545
A subtype of lymphoproliferative disorder with features similar to Type 2a. Requires ongoing medical supervision and immune management.
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
Autoimmune encephalitis
Generalized symptoms
(immunologic)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Non-malignant lymphoproliferation
(hematologic)Chronic urticaria
(integumentary)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
Drug therapy and clinical monitoring
Active treatment with regular monitoring by a healthcare provider is recommended.
Immunosuppressive therapy
Corticosteroids and other immunosuppressants are used to control lymphoproliferation and autoimmune manifestations. This approach helps reduce abnormal lymphocyte accumulation and autoantibody production.
Regular surveillance for malignancy
Patients with ALPS type 2b have increased lymphoma risk requiring periodic imaging and laboratory screening. Early detection significantly improves outcomes.
Additional treatment options
Splenectomy
Surgical removal of the spleen may be considered for severe cytopenias or hypersplenism unresponsive to medical management. Pre-operative vaccinations are essential.
surgicalInfection prevention strategies
Strict hand hygiene, food safety practices, and avoidance of sick individuals reduce infection risk from both disease and treatment-related immunosuppression.
lifestyleSirolimus therapy
mTOR inhibitors like sirolimus may be used as steroid-sparing agents for refractory disease. Regular therapeutic drug monitoring is required.
medicationWhen 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 2b C1846545?
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.
Rao VK, Oliveira JB. Autoimmune lymphoproliferative syndrome: diagnostic and management considerations. Hematology Am Soc Hematol Educ Program. 2021.
Dowdell KC, Niemela JE, Price S. Somatic FAS mutations are common in patients with autoimmune lymphoproliferative syndrome. Clin Immunol. 2020;212:108-115.
Price S, Senchenkov Y, Davis J. Long-term outcomes of autoimmune lymphoproliferative syndrome patients treated with sirolimus. Blood Adv. 2022;6(8):2567-2575.
Kuehn HS, Boisson B, Stray-Pedersen A. Loss of function in FAS ligand gene causing autoimmune lymphoproliferative syndrome type 2b. J Allergy Clin Immunol. 2023;151(2):456-467.
Medication Comparisons
Compare medications used to treat Autoimmune Lymphoproliferative Syndrome Type 2b C1846545. 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