Pentostatin for Treatment of Refractory Autoimmune Lymphoproliferative Syndrome

被引:8
|
作者
Bajwa, Rajinder [1 ]
Savelli, Stephanie [2 ]
Gross, Thomas [1 ]
机构
[1] Nationwide Childrens Hosp, Dept Hematol Oncol BMT, Columbus, OH 43205 USA
[2] Childrens Hosp Med Ctr Akron, Akron, OH USA
关键词
autoimmune cytopenias; autoimmune lymphoproliferative syndrome; pentostatin; refractory ALPS; MYCOPHENOLATE-MOFETIL; SYNDROME ALPS;
D O I
10.1002/pbc.23144
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
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. (C) 2011 Wiley-Liss, Inc.
引用
收藏
页码:336 / 337
页数:2
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