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Case Reports in Hematology
Volume 2016, Article ID 9515404, 3 pages
http://dx.doi.org/10.1155/2016/9515404
Case Report

Primary Myelofibrosis Presenting as Extramedullary Hematopoiesis in a Transplanted Liver Graft: Case Report and Review of the Literature

1Department of Internal Medicine, Kansas University Medical Center, 3901 Rainbow Boulevard, Kansas City, KS 66103, USA
2Department of Hematology-Oncology, Kansas University Medical Center, 3901 Rainbow Boulevard, Kansas City, KS 66103, USA

Received 3 September 2015; Revised 7 December 2015; Accepted 29 December 2015

Academic Editor: Eduardo Arellano-Rodrigo

Copyright © 2016 Ghulam Rehman Mohyuddin and Abdulraheem Yacoub. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Primary myelofibrosis (PMF) commonly results in extramedullary hematopoiesis (EMH) in the spleen and liver as well as a variety of other organs. We present a first report of a unique presentation of PMF in a liver transplant recipient patient as EMH in the transplanted liver graft. A 76-year-old man with history of cryptogenic cirrhosis received cadaveric liver transplantation in 1996. He maintained a normal graft function and stable hematologic parameters until 2013 when he presented with anemia and progressive fatigue. Extensive work-up did not identify the etiology of the recent decline in his hemoglobin; thus a liver biopsy was done which showed findings of EMH within the sinusoids with increased megakaryocytes, some with atypical morphology. A BM biopsy revealed a hypercellular marrow, moderately increased reticulin fibrosis, and features consistent with primary myelofibrosis. Abdominal imaging showed a normal-size spleen and did not identify any sites of EMH outside of the liver. The diagnosis of myelofibrosis was thus made, and this case demonstrated predominant tropism to a transplanted liver graft with absence of EMH elsewhere. We would thus like to emphasize that findings of EMH in subjects with no preexisting hematologic neoplasm should warrant close follow-up and assessment.