Secondary Myelofibrosis Diagnosis

In 2008, the diagnostic criteria for myeloproliferative neoplasms (MPNs) were revised by the World Health Organization (WHO) and the International Working Group for Myelofibrosis Research and Treatment (IWG-MRT).1,2 Diagnostic information for post–polycythemia vera myelofibrosis (post-PV MF) and post–essential thrombocythemia myelofibrosis (post-ET MF) is below.

The presence or absence of the JAK2V617F mutation does not independently determine the diagnosis of myelofibrosis.1

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2008 IWG-MRT Diagnostic Criteria for Post-PV MF and Post-ET MF

ET = essential thrombocythemia; LDH = lactate dehydrogenase; MF = myelofibrosis; PV = polycythemia vera.

aGrade 2-3 according to the European classification: Diffuse, often coarse fiber network with no evidence of collagenization (negative trichrome stain) or diffuse, coarse fiber network with areas of collagenization (positive trichrome stain). Grade 3-4 according to the standard classification: Diffuse and dense increase in reticulin with extensive intersections, occasionally with only focal bundles of collagen and/or focal osteosclerosis or diffuse and dense increase in reticulin with extensive intersections with coarse bundles of collagen, often associated with significant osteosclerosis.2

bBelow the reference range for appropriate age, sex and altitude considerations.2

References:

  1. Tefferi A, Vardiman JW. Classification and diagnosis of myeloproliferative neoplasms: the 2008 World Health Organization criteria and point-of-care diagnostic algorithms. Leukemia. 2008;22:14-22.
  2. Barosi G, Mesa RA, Thiele J, et al; International Working Group for Myelofibrosis Research and Treatment (IWG-MRT). Proposed criteria for the diagnosis of post-polycythemia vera and post-essential thrombocythemia myelofibrosis: a consensus statement from the International Working Group for Myelofibrosis Research and Treatment. Leukemia. 2008;22:437-438.
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