Autoimmune lymphoproliferative syndrome history and symptoms

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Editor-In-Chief: David Teachey, MD [1] Associate Editor(s)-in-Chief: Sharmi Biswas, M.B.B.S

Overview

Autoimmune lymphoproliferative syndrome (ALPS) hard to diagnose due to expressions of different phenotypes and overlapping symptoms with other hematological disorders. The most common symptoms of ALPS are related to lymphadenopathy predominantly in cervical region, splenomegaly with or without features of hypersplenism or hepatomegaly, and autoimmune cytopenias as thrombocytopenia, hemolytic anemia or occasional neutropenia. Autoimmune cytopenia and lymphoproliferation occur simultaneously in most cases but can also happen separately or in an interval.

Common symptoms include:

↑ Rieux-Laucat, Frédéric; Magérus-Chatinet, Aude; Neven, Bénédicte (2018). "The Autoimmune Lymphoproliferative Syndrome with Defective FAS or FAS-Ligand Functions". Journal of Clinical Immunology. 38 (5): 558–568. doi:10.1007/s10875-018-0523-x. ISSN 0271-9142.
↑ Matson, Daniel R.; Yang, David T. (2019). "Autoimmune Lymphoproliferative Syndrome: An Overview". Archives of Pathology & Laboratory Medicine. 144 (2): 245–251. doi:10.5858/arpa.2018-0190-RS. ISSN 0003-9985.