Chronic neutrophilic leukemia 2014: Update on diagnosis, molecular genetics, and management. Issue 6 (May 2014)

• Record Type: Journal Article
• Authors: Elliott, Michelle A.; Tefferi, Ayalew
• Is Part Of: American journal of hematology. Volume 89:Issue 6(2014:Jun.)
• Page Start: 651
• Page End: 658
• Publication Date: 2014-05
• Subjects: Hematology -- Periodicals; 616.15
• DOI: 10.1002/ajh.23667
• Languages: English
• ISSNs: 0361-8609

Abstract

<abstract abstract-type="main"> <title> <x xml:space="preserve">Abstract</x> </title> <p> <bold><italic>Disease Overview</italic></bold>: Chronic neutrophilic leukemia (CNL) is a myeloproliferative neoplasm characterized by sustained, mature neutrophilic leukocytosis, splenomegaly, and bone marrow granulocytic hyperplasia.</p> <p> <bold><italic>Diagnosis</italic></bold>: Key diagnostic criteria include leukocytosis of <underline>&gt;</underline>25 × 10⁹/l (of which &gt;80% are neutrophils) with &lt;10% and &lt;1% circulating immature granulocytes and myeloblasts, respectively. There should be no dysplasia, monocytosis, molecular evidence of <italic>BCR‐ABL1</italic>, <italic>PDGFRA</italic>, <italic>PDGFRB</italic>, or <italic>FGRF1</italic> rearrangements and no identifiable cause for physiologic neutrophilia or, if present, demonstration of myeloid clonality.</p> <p> <bold><italic>Developments in Molecular Genetics</italic></bold>: Recently, CNL has been shown to be specifically driven by somatic activating mutations of <italic>CSF3R</italic>, most commonly <italic>CSF3R</italic> 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 <italic>CSF3R</italic>. Am. J. Hematol. 89:651–658, 2014. © 2014 Wiley Periodicals, Inc.</p> </abstract>