Autoimmune Lymphoproliferative Syndrome (ALPS) in a Boy with Massive Lymphadenopathy

Autoimmune lymphoproliferative syndrome (ALPS) is an uncommon nonmalignantlymphoproliferative disease which is characterized by chronic, persistent or recurrentlymphadenopathy, splenomegaly, hepatomegaly, immune cytopenia, hypergammaglobinemia and increased risk of lymphoma.We report a 2-year old boy with hepatosplenomegaly as first presentation. Petechial andpurpuric rashes with massive cervical lymphadenopathies developed 10 months later. Inlaboratory tests anemia, thrombocytopenia and hypergammaglobinemia were observed.According to flocytometry increased double negative T cells and by apoptosis assay decrease apoptosis of lymphocytes accompanied clinical manifestations, thus diagnosis of ALPS was established.In conclusion; in all patients with massive lymphadenopathy and hepatosplenomegay;especially with cytopenia; ALPS should be considered.