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Pentostatin for treatment of refractory autoimmune lymphoproliferative syndrome
Author(s) -
Bajwa Rajinder,
Savelli Stephanie,
Gross Thomas
Publication year - 2011
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.23144
Subject(s) - medicine , autoimmune lymphoproliferative syndrome , pentostatin , splenectomy , autoimmune hemolytic anemia , neutropenia , immunology , hematopoietic stem cell transplantation , blood cancer , evans syndrome , autoimmune disease , lymphoma , transplantation , cancer , rituximab , antibody , apoptosis , chemotherapy , spleen , programmed cell death , fas receptor , biochemistry , chemistry
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). Pediatr Blood Cancer 2011; 57: 336–337. © 2011 Wiley‐Liss, Inc.