Autoimmune lymphoproliferative syndrome classification

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.

The revised classification of ALPS is as following ^[1]
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

↑ Oliveira, Joao B.; Bleesing, Jack J.; Dianzani, Umberto; Fleisher, Thomas A.; Jaffe, Elaine S.; Lenardo, Michael J.; Rieux-Laucat, Frederic; Siegel, Richard M.; Su, Helen C.; Teachey, David T.; Rao, V. Koneti (2010). “Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS): report from the 2009 NIH International Workshop”. Blood. 116 (14): e35–e40. doi:10.1182/blood-2010-04-280347. ISSN 0006-4971.