Research progress of autoimmune lymphoproliferative syndrome / 中华实用儿科临床杂志

ABSTRACT

Autoimmune lymphoproliferative syndrome (ALPS) is a human genetic disorder of lymphocyte apoptosis resulting in an accumulation of lymphocytes and childhood onset chronic lymphadenopathy, splenomegaly, multilineage cytopenias, and an increased risk of B-cell lymphoma.Since then, with more than 500 patients with ALPS diagnosed worldwide.In this paper, it is hoped that harmonizing the diagnosis and classification of ALPS will foster be-tter understanding of the pathogenesis of ALPS.