Autoimmune lymphoproliferative syndrome (ALPS): A case with congenital onset

被引：5
|
作者：
Kahwash, Samir B.
Fung, Bonita
Savelli, Stephanie
Bleesing, Jack J.
Qualman, Stephen J.
机构：
[1] Childrens Hosp, Dept Lab Med, Columbus, OH 43205 USA
[2] Childrens Hosp, Dept Pediat, Hematol Oncol Sect, Columbus, OH 43205 USA
[3] Childrens Hosp, Med Ctr, Dept Pediat, Div Hematol Oncol, Cincinnati, OH 45229 USA
来源：
PEDIATRIC AND DEVELOPMENTAL PATHOLOGY | 2007年 / 10卷 / 04期
关键词：
autoimmune lymphoproliferative syndrome (ALPS); bone marrow transplant; congenital; double-negative T cells;
D O I：
10.2350/06-06-0105.1
中图分类号：
R36 [病理学];
学科分类号：
100104 ;
摘要：
We describe a case of autoimmune lymphoproliferative syndrome (ALPS), which is very unusual with regard to a clinical onset soon after birth, and a clinical picture dominated by splenomegaly, jaundice, and consumptive peripheral blood cytopenias, with minimal lymphadenopathy. Our documented close follow up demonstrated initial involvement of the spleen, followed by involvement of the bone marrow and the peripheral blood. The patient underwent bone marrow transplant and is alive and well 20 months after diagnosis.
引用
收藏
页码：315 / 319
页数：5
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