Autoimmune lymphoproliferative syndrome classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: David Teachey, MD [2] Sharmi Biswas, M.B.B.S

Overview

Classification of ALPS is based on the recommendations made by first international ALPS workshop held at National Institutes of Health in 2009.

Classification

The revised classification of ALPS is as following

Previous nomenclature Revised nomenclature Gene Definition

ALPStype IIb CEDS CASP8 Splenomegaly, marginal raised DNT, recurrent infections, germline mutations in caspase 8

ALPS type IV RALD NRAS Autoimmunity, lymphadenopathy and/or splenomegaly, elevated or normal DNTs, somatic mutations in NRAS

DALD DALD Unknown Lymphadenopathy and /or splenomegaly, autoimmunity, normal DNTs, defective in vitro FAS-mediated apoptosis

XLP1 XLP1 SH2D1A Hypogammaglobulinemia, fulminant Epstein- Barr virus infection, or lymphoma