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Radiological Imaging in Hematological Malignancies pp 389–408Cite as

Myelofibrosis

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Part of the book series: Medical Radiology ((Med Radiol Diagn Imaging))

Abstract

Myelofibrosis, also called myeloid metaplasia, is a myeloproliferative disorder characterized by progressive bone marrow fibrosis, splenomegaly, extramedullary hematopoiesis, leukoerythroblastosis, anisopoikilocytosis, and teardrop poikilocytes. Most patients have moderate to severe anemia. Bone marrow in patients with myelofibrosis is usually hypocellular, with increased amounts of reticulin and collagen. Myelofibrosis may be primary (or idiopathic) or secondary, and usually affects adults, with only a few reports in children (Guermazi et al. 1999). Myelofi brosis is of unknown etiology, but association with an autoimmune diseases such as polyarteritis nodosa (Camos et al. 2003), ulcerative colitis (Arellano-Rodrigo et al. 2002), or primary biliary cirrhosis (Hernandez-Boluda et al. 2002) gives support to the hypothesis of an immune basis of some idiopathic myelofibrosis cases. The average life expectancy of patients after diagnosis is 2 to 3 years. Only 8% of patients with the disease survive from 6 to 8 years. The common imaging findings in patients with myelofibrosis are osteosclerosis, hepatosplenomegaly, and lymphadenopathies. In addition, extramedullary hematopoiesis may develop in multiple sites such as chest, abdomen, pelvis, and central nervous system, simulating malignant disease (Guermazi et al. 1999). The purpose of this chapter is to illustrate the wide range of radiological abnormalities in patients affected by this serious, progressive and fatal disease.

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  1. Department of Radiology, University of California, San Francisco, 350 Parnassus Avenue, Suite 150, San Francisco, CA, 94117, USA

    Ali Guermazi

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  1. Ali Guermazi
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  1. Radiographic Laboratory Osteporosis & Arthritis Research Group, University of California, San Francisco, 350 Pernassus Avenue, Suite 150, San Francisco, CA, 94117, USA

    Ali Guermazi MD (Visiting Associate Professor Department of Radiology, Director) (Visiting Associate Professor Department of Radiology, Director)

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© 2004 Springer-Verlag Berlin Heidelberg

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Guermazi, A. (2004). Myelofibrosis. In: Guermazi, A. (eds) Radiological Imaging in Hematological Malignancies. Medical Radiology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-18832-9_21

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  • DOI: https://doi.org/10.1007/978-3-642-18832-9_21

  • Publisher Name: Springer, Berlin, Heidelberg

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