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autoimmune lymphoproliferative syndrome

Medical Definition

Rare congenital lymphoid disorder due to mutations in certain fas-fas ligand pathway genes. known causes include mutations in fas, tnfsf6, nras, casp8, and casp10 proteins. clinical features include lymphadenopathy; splenomegaly; and autoimmunity.

Wikipedia Summary

Autoimmune lymphoproliferative syndrome (ALPS) is a form of lymphoproliferative disorder (LPDs). It affects lymphocyte apoptosis. It is a rare genetic disorder of abnormal lymphocyte survival caused by defective Fas mediated apoptosis...
Related Codes (1)
Code
Description
Billable
Details
D89.82Autoimmune lymphoproliferative syndrome [ALPS]

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