Hepatitis-associated aplastic anemia presenting as a familial bone marrow failure syndrome.
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 postliver transplantation. In the process of family screening to locate a donor for hematopoietic stem 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.
Breakey
Vicky Rowena
VR
Department of Pediatrics, Division of Pediatric Hematology and Oncology, Research Institute, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
Meyn
Stephen
S
Ng
Vicky
V
Allen
Christopher
C
Dokal
Inderjeet
I
Lansdorp
Peter M
PM
Abla
Oussama
O
Dror
Yigal
Y
D000741:Anemia, Aplastic / Q000175:diagnosis / Q000209:etiology; D002648:Child; D003937:Diagnosis, Differential; D005260:Female; D006505:Hepatitis / Q000150:complications / Q000175:diagnosis / Q000601:surgery; D006801:Humans; D017114:Liver Failure, Acute / Q000150:complications / Q000175:diagnosis / Q000601:surgery; D016031:Liver Transplantation; D008297:Male; D009190:Myelodysplastic Syndromes / Q000175:diagnosis / Q000209:etiology; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis / Q000209:etiology; D035781:Siblings; D014184:Transplantation, Homologous