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

Breakey, Vicky Rowena MD*; Meyn, Stephen MD, PhD; Ng, Vicky MD; Allen, Christopher BSc; Dokal, Inderjeet MD§; Lansdorp, Peter M. PhD; Abla, Oussama MD*; Dror, Yigal MD* †

Journal of Pediatric Hematology/Oncology: November 2009 - Volume 31 - Issue 11 - p 884-887
doi: 10.1097/MPH.0b013e3181b86ec3
Clinical and Laboratory Observations
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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.

*Department of Pediatrics, Division of Pediatric Hematology and Oncology

Cell Biology Program, Research Institute, the Hospital for Sick Children, the University of Toronto

Division of Genetics and Division of Gastroenterology and Nutrition, The Hospital for Sick Children

Terry Fox Laboratory, British Columbia Cancer Agency and University of British Columbia, Vancouver, British Columbia, Toronto, Ontario, Canada

§Centre for Paediatrics, Institute of Cell and Molecular Science, Barts and the London School of Medicine and Dentistry, London, UK

The Canadian Inherited Marrow Failure Registry was supported by grants from the C17 Research Network and Cancer Candlelighters Canada, the Fanconi Anemia Association of Canada, the Neutropenia Association of Canada and Shwachman-Diamond Syndrome Canada.

Reprints: Yigal Dror, MD, Department of Pediatrics, Division of Haematology and Oncology, Cell Biology Program, Research Institute, the Hospital for Sick Children 555 University Avenue, Toronto, Ontario, M5G1×8, Canada (e-mail: yigal.dror@sickkids.ca).

Received for publication October 8, 2008

accepted July 18, 2009

Conflict of Interest Disclosure: Peter M. Lansdorp is a founding shareholder in Repeat Diagnostics Inc, a company specializing in leukocyte telomere length measurements using flow FISH. All other authors declare no competing financial interests.

© 2009 Lippincott Williams & Wilkins, Inc.