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Autoimmune lymphoproliferative syndrome
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Autoimmune lymphoproliferative syndrome

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Synopsis

Autoimmune lymphoproliferative syndrome (ALPS) is a rare disease caused by a mutation of genes responsible for monitoring programmed cell death (apoptosis). ALPS most commonly occurs as a result of FAS (CD95) gene mutations, although other pathways have been identified. Typically, a child first presents with lymphoproliferative symptoms of lymphadenopathy and splenomegaly and years later with autoimmune symptoms of hemolytic anemia and thrombocytopenia. Patients with the classic form of ALPS (type 1A) are largely predisposed to developing Hodgkin and non-Hodgkin lymphomas. There is currently no cure for ALPS, but management consists of treating cytopenias and any co-occurring diseases.

For more information, see OMIM.

Codes

ICD10CM:
D89.82 – Autoimmune lymphoproliferative syndrome [ALPS]

SNOMEDCT:
702444009 – Autoimmune lymphoproliferative syndrome

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Last Updated: 03/29/2017
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Autoimmune lymphoproliferative syndrome
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Autoimmune lymphoproliferative syndrome : Hepatomegaly, Hemolytic anemia, Lymphadenopathy, Splenomegaly, Hives , WBC decreased, PLT decreased, LYP decreased
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