A case of autoimmune lymphoproliferative syndrome in a 3 years-old patient
نویسندگان
چکیده
Background Autoimmune lymphoproliferative syndrome (ALPS) a disease which is based on primary violations lymphocyte apoptosis caused by various molecular defects: mutation in the proapoptotic receptor Fas (la ALPS type) or its ligand FasL (Ib type), a mutation in the gene caspase10 (type II), or an unidentified defect in the signaling pathway of Fas-receptor (III type). The typical clinical picture: a non-cancerous chronic lymphadenopathy, splenomegaly, autoimmune defects, cytopenias, hypergammaglobulinemia with high serum IgA and increasing in the number of double negative TCR-alfa-betaCDZ + CD4-CD8-lymphocytes.
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