Hepatitis-associated Aplastic Anemia Presenting as a Familial Bone Marrow Failure Syndrome

被引:5
作者
Breakey, Vicky Rowena [2 ]
Meyn, Stephen
Ng, Vicky [3 ,4 ]
Allen, Christopher
Dokal, Inderjeet [7 ]
Lansdorp, Peter M. [5 ,6 ]
Abla, Oussama [2 ]
Dror, Yigal [1 ,2 ]
机构
[1] Univ Toronto, Hosp Sick Children, Dept Pediat, Div Haematol & Oncol,Cell Biol Program,Res Inst, Toronto, ON M5G 1X8, Canada
[2] Univ Toronto, Hosp Sick Children, Div Pediat Hematol & Oncol, Dept Pediat, Toronto, ON M5G 1X8, Canada
[3] Hosp Sick Children, Div Genet, Toronto, ON M5G 1X8, Canada
[4] Hosp Sick Children, Div Gastroenterol & Nutr, Toronto, ON M5G 1X8, Canada
[5] British Columbia Canc Agcy, Terry Fox Lab, Toronto, ON, Canada
[6] Univ British Columbia, Toronto, ON, Canada
[7] Barts & London Queen Marys Sch Med & Dent, Inst Cell & Mol Sci, Ctr Paediat, London, England
关键词
bone marrow failure syndrome; aplastic anemia; fulminant hepatic failure; myelodysplastic syndrome; acute lymphoblastic leukemia; inherited; ACUTE LIVER-FAILURE; NON-A; NON-B; 3-YEAR-OLD BOY; TRANSPLANTATION; CHILDREN; MUTATIONS; DYSFUNCTION; GENE;
D O I
10.1097/MPH.0b013e3181b86ec3
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
Hepatitis-associated aplastic anemia is a well-described entity after idiopathic fulminant hepatic failure. The hematologic disease ranges from mild-to-severe aplastic anemia and the cause of the disease is unknown. We describe 2 siblings with bone marrow failure. The older child presented with idiopathic fulminant hepatic failure and an early onset of rapidly progressive severe aplastic anemia that developed into myelodysplastic syndrome post-liver transplantation. In the process of family screening to locate a donor for hematopoietic stein cell transplantation, the younger sibling was found to have hypocellular bone marrow and later developed acute lymphoblastic leukemia. These familial cases raise the possibility of an inherited bone marrow failure syndrome and suggest that severe hepatitis-associated aplastic anemia may not be always an acquired condition.
引用
收藏
页码:884 / 887
页数:4
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