%0 Journal Article
%T A Case of Philadelphia Chromosome Positive Myeloproliferative Neoplasm in a Pregnant Woman with Unusual Primary Myelofibrosis Features
%A Jason Koshy
%A Jack Alperin
%A Bagi Jana
%A Avi Markowitz
%A You-Wen Qian
%J Case Reports in Hematology
%D 2013
%I Hindawi Publishing Corporation
%R 10.1155/2013/702831
%X Myeloproliferative neoplasms (MPNs) are traditionally separated into BCR-ABL-positive chronic myeloid leukemia (CML), and BCR-ABL-negative MPNs including primary myelofibrosis (PMF), essential thrombocythemia (ET), and so forth. One of the diagnostic requirements for PMF and ET is the absence of the Philadelphia chromosome, while its presence is almost universally indicative of CML. However, a diagnostic dilemma arises when Philadelphia chromosome-positive MPNs lack the majority of the typical features seen in CML. Some of these classic CML features include basophilIa, marked leukocytosis, neutrophils left-shift with myelocytes bulge, and ¡°dwarf¡± megakaryocytes. Presented here is a case of a 32-year-old pregnant patient who did not have typical morphologic findings for CML, and yet the Philadelphia chromosome was positive. The patient demonstrated some pathologic features that are commonly presented in PMF that included bone marrow reticulin fibrosis, leukoerythroblastosis, splenomegaly, and increased serum lactate dehydrogenase. 1. Introduction Primary myelofibrosis (PMF) is a distinct, well-defined myeloproliferative neoplasm (MPN) characterized by megakaryocytic hyperplasia and subsequent fibrosis in the bone marrow, as well as leukoerythroblastosis and organomegaly. The presence of the , also known as the Philadelphia chromosome translocation, is used to demarcate chronic myelogenous leukemia (CML) from other MPNs including PMF. We describe an unusual case of a 32-year-old pregnant patient who presented with thrombocytosis and was found to have Philadelphia chromosome. The overall clinical presentation in this patient including bone marrow features were more commonly seen in a PMF, although other MPNs such as essential thrombocythemia (ET) were also considered. 2. Case Presentation The patient was a 32-year-old gravid 2 para 1 patient at 8 weeks gestation who presented to her obstetrician for diabetes management. She had a history of thrombocytosis in 2010 and was prescribed Plavix by her physician at an outside hospital. She denied any history of thrombosis but did complain of bleeding when she brushed her teeth. The physical examination was significant for hepatosplenomegaly which was later confirmed with abdominal imaging (Figure 1). Initial complete blood count (CBC) revealed a platelet count of 1,291 ¡Á 109/L (reference range 166¨C358 ¡Á 109/L), hemoglobin of 10.7£¿g/dL (reference range 11.6¨C15£¿g/dL), hematocrit 33.4% (reference range 35.7¨C45.2%), and a white blood cell (WBC) count of 15.7 ¡Á 109/L (reference range 4.3¨C11.1 ¡Á 109/L) with no
%U http://www.hindawi.com/journals/crihem/2013/702831/