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Is it chronic idiopathic myelofibrosis, myelofibrosis with myeloid metaplasia, chronic megakaryocytic–granulocytic myelosis, or chronic megakaryocytic leukemia? Further thoughts on the nosology of the clonal myeloid disorders

Leukemia volume 19, pages 1139–1141 (2005)Cite this article

The designations given to the clonal (neoplastic) lymphohematopoietic disorders undergo periodic revision. The World Health Organization (WHO) draws together experts to consider reclassification of these diseases as knowledge of their pathobiology advances.1 The clonal lymphoid diseases have had notable attention. These neoplasms arise from transforming mutations in lymphocytes that have been defined with increasing precision. They have been related to their presumptive normal homologue in the lymphocyte maturation sequence and to the localization of their normal homologue in a relevant lymphoid structure (eg, skin, gut region, lymph node region, spleen, etc). The application of gene expression analysis will result in further refinements of the classification. The next iteration of the classification of the clonal lymphoid diseases will include at least two and, perhaps, three subtypes of diffuse large B-cell lymphoma. It is likely that other refinements in classification based on gene expression profiles will be made. Three cheers for the lymphologists!

The re-evaluation of the classification of the clonal myeloid diseases has not been as frequent as that of the clonal lymphoid diseases, although a revision was published in 2001.1 In general, the changes were minimal in that obsolescent terminology such as ‘refractory anemia’ was left in place and the phrase ‘excess blasts’, which is a reflection of fuzzy thinking, was perpetuated. Also, the classification was made redundant by listing morphologic and genetic alterations that are not mutually exclusive. A practical and comprehensive subclassification of the clonal myeloid diseases by genotype is unlikely to replace completely the morphologic classification because of the large number of apparent primary genetic mutations associated with myeloid diseases. For example, there have been over 100 primary genetic alterations associated with AML alone, although three major translocations, (t(8;21), t(15;17), Inv16 or t(16;16)), account for about 20% of cases, if one includes all age groups.

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  1. University of Rochester Medical Center, Rochester, NY, USA

    M A Lichtman

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Lichtman, M. Is it chronic idiopathic myelofibrosis, myelofibrosis with myeloid metaplasia, chronic megakaryocytic–granulocytic myelosis, or chronic megakaryocytic leukemia? Further thoughts on the nosology of the clonal myeloid disorders. Leukemia 19, 1139–1141 (2005). https://doi.org/10.1038/sj.leu.2403804

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