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Description of serologic features in autoimmune lymphoproliferative syndrome

BACKGROUND: Autoimmune lymphoproliferative syndrome (ALPS) is a recently recognized and rare disorder associated with inherited defects in the Fas gene or other regulators of lymphocyte apoptosis. It is characterized by massive lymphadenopathy; splenomegaly; autoimmunity including episodes of immune hemolytic anemia, thrombocytopenia, and neutropenia. 1 The serologic basis for immune cytopenias associated with ALPS has not been previously characterized.