pentostatin and Autoimmune-Lymphoproliferative-Syndrome

Topics: Antineoplastic Agents; Autoimmune Lymphoproliferative Syndrome; Diseases in Twins; Humans; Male; Pentostatin

Autoimmune lymphoproliferative syndrome (ALPS), a disorder of programmed cell death, could be due to a congenital defect in the Fas signaling pathway or other pathways for apoptosis. Most cases present with lymphoproliferation and certain autoimmune features such as thrombocytopenia, neutropenia, and anemia are due to excessive production of antibodies by B lymphocytes. Majority of cases present within the first few years of life. We report a case of ALPS presenting at birth which was refractory to splenectomy and immunosuppressive therapy, but responded to pentostatin followed by hematopoietic stem cell transplantation (HSCT).

Topics: Antineoplastic Agents; Autoimmune Lymphoproliferative Syndrome; Combined Modality Therapy; Diseases in Twins; Hematopoietic Stem Cell Transplantation; Humans; Infant, Newborn; Male; Pentostatin