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Chronic neutrophilic leukemia 2014: Update on diagnosis, molecular genetics, and management
Author(s) -
Elliott Michelle A.,
Tefferi Ayalew
Publication year - 2014
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.23667
Subject(s) - neutrophilia , monocytosis , leukocytosis , pdgfrb , myeloproliferative neoplasm , pdgfra , medicine , myeloid , myeloproliferative disorders , bone marrow , immunology , cancer research , biology , myelofibrosis , genetics , stromal cell , gene , gist
Disease Overview : Chronic neutrophilic leukemia (CNL) is a myeloproliferative neoplasm characterized by sustained, mature neutrophilic leukocytosis, splenomegaly, and bone marrow granulocytic hyperplasia. Diagnosis : Key diagnostic criteria include leukocytosis of > 25 × 10 9 /l (of which >80% are neutrophils) with <10% and <1% circulating immature granulocytes and myeloblasts, respectively. There should be no dysplasia, monocytosis, molecular evidence of BCR‐ABL1 , PDGFRA , PDGFRB , or FGRF1 rearrangements and no identifiable cause for physiologic neutrophilia or, if present, demonstration of myeloid clonality. Developments in Molecular Genetics : Recently, CNL has been shown to be specifically driven by somatic activating mutations of CSF3R , most commonly CSF3R T618I. As such, the diagnosis of CNL will no longer be one of exclusion only, and revision of the current WHO classification is anticipated to include the molecular criterion of mutated CSF3R . Am. J. Hematol. 89:651–658, 2014. © 2014 Wiley Periodicals, Inc.