Autoimmune Lymphoproliferative Syndrome; A Case Report

Autoimmune lymphoproliferative syndrome is a disorder of lymphoid systemregulation characterized by chronic splenomegaly, lymphadenopathy and autoimmunephenomena especially immune-mediated cytopenias. the hallmark of the disease is thepresence in peripheral blood and lymphoid tissue of increased numbers of a normallyrare t lymphocyte subset, usually referred to as “double-negative” t cells. here theauthors report a 16-year-old boy when he was first hospitalized for diffuse petechiae,purpura and epistaxis at 9 years of age.one year later,he was readmitted for high feverand recurring cytopenia. on examination several enlarged, nontender lymph nodesinvolving cervical and submandibular areas and a huge spleen were detected.lymphnode biopsy was performed two times. according to flowcytometry of peripheral bloodand immunophenotyping of lymph node tissues which revealed increased numbers ofcd3+cd4-cd8- t-lymphocytes autoimmune lymphoproliferative syndrome wassuggested for him. autoimmune lymphoproliferative syndrome should be consideredin differential diagnosis of any patient with unexplained coomb’s positive cytopenias,hypergammaglobulinemia, generalized lymphadenopathy and splenomegaly. theconfirmation of the diagnosis should be based upon genetic analysis and detection ofthe affected genes involved in fas pathway