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Myeloproliferative Disorders

  • S. Smith
  • W. Stock
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Abstract

The chronic myeloproliferative disorders (CMDs) are characterized by clonal hematopoietic stem cell expansion along with overproduction of one or more blood cell lines. CMDs can be divided into two categories based on the presence or absence of a fusion gene, bcr-abl, resulting from the chromosomal translocation between chromosomes 9 and 22. Any CMD with the bcr-abl translocation, either cytogenetically or molecularly determined, is categorized as chronic myelogenous leukemia and is discussed separately (Chap. 15, “Chronic Myelogenous Leukemia”). The bcr-abl-negative disorders include the typical chronic myeloproliferative disorders of polycythemia vera (PV), essential thrombocythemia (ET) myelofibrosis with myeloid metaplasia (MMM), and the atypical CMDs of chronic neutrophilic leukemia, mast cell disease and chronic eosinophilic leukemia [1]. This last group of disorders is extremely rare and is not considered in this review. Although PV, ET, and MMM are distinct entities, they can have overlapping clinical and laboratory features. The terms postthrombocythemic myeloid metaplasia (PTMM) and postpolycythemic myeloid metaplasia (PPMM) refer to the progression of ET and PV, respectively, to a clinical scenario of MMM [2].

Keywords

Polycythemia Vera Essential Thrombocythemia Thrombotic Complication Myeloproliferative Disorder Leukemic Transformation 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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© Springer-Verlag Berlin Heidelberg 2003

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  • S. Smith
  • W. Stock

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