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WHO-defined chronic neutrophilic leukemia: a long-term analysis of 12 cases and a critical review of the literature

Leukemia volume 19, pages 313–317 (2005)Cite this article

TO THE EDITOR,

Chronic neutrophilic leukemia (CNL) is a rare chronic myeloproliferative disorder (CMPD) with only ∼150 cases reported to date, which has only recently attained formal recognition as a distinct entity within the updated World Health Organization (WHO) classification system.1, 2 CNL is characterized by sustained, mature neutrophilic leukocytosis with few or no circulating immature granulocytes, monocytosis or basophilia. Affected patients usually display splenomegaly and bone marrow (BM) histology reveals granulocytic hyperplasia without evidence of dysplasia or striking reticulin fibrosis. Cytogenetic and molecular analyses are negative for the Philadelphia chromosome (Ph chromosome) and/or its molecular counterpart, fusion of bcr and abl genes (b2/a2, b3/a2 or e19/a2 junction). The diagnosis is contingent upon thorough initial investigation and follow-up to exclude the underlying causes of reactive neutrophilia, particularly if evidence of myeloid clonality is lacking. In addition, features of other CMPD, chronic myelogenous leukemia (CML), atypical CML (aCML), and chronic myelomonocytic leukemia (CMML), as defined by the WHO, must be absent.2 Furthermore, CNL should be differentiated from neutrophilic-CML (CML-N), which is a rare variant of bcr/abl (e19/a2 junction)-driven Ph-positive CML.

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Authors and Affiliations

  1. Department of Internal Medicine, Division of Hematology, Mayo Clinic, Rochester, MN, USA

    M A Elliott, C A Hanson, G W Dewald, S A Smoley, T L Lasho & A Tefferi

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  1. M A Elliott
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  2. C A Hanson
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Correspondence to M A Elliott.

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Elliott, M., Hanson, C., Dewald, G. et al. WHO-defined chronic neutrophilic leukemia: a long-term analysis of 12 cases and a critical review of the literature. Leukemia 19, 313–317 (2005). https://doi.org/10.1038/sj.leu.2403562

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