Chronic neutrophilic leukemia 2016: Update on diagnosis, molecular genetics, prognosis, and management

Michelle A. Elliott, Ayalew Tefferi

Research output: Contribution to journalArticlepeer-review

17 Scopus citations


Chronic neutrophilic leukemia (CNL) is a potentially aggressive myeloproliferative neoplasm, for which current WHO diagnostic criteria include leukocytosis of ≥25 × 109/L (of which >80% are neutrophils) and with <10 and <1% circulating immature granulocytes and blasts, respectively without dysplasia, clinical, or molecular criteria for other myeloproliferative disorders, nor an identifiable cause for physiologic neutrophilia in the absence of markers of myeloid clonality. Such a pathogenic clonal marker has now been identified as a somatic activating mutation of CSF3R, most commonly CSF3R T618I, thus demanding revision of the current WHO diagnostic classification to include the molecular criterion of mutated CSF3R. The clinical presentation, disease course and prognosis of CSF-R mutated CNL have been recently outlined. Co-operative mutations in SETBP1 and ASXL1 appear to be of prognostic significance and correlate with disease progression. Advances in the understanding of the molecular pathogenesis of CNL, have not yet fully translated into satisfactory therapeutic strategies, but the foundations for these are strengthening.

Original languageEnglish (US)
Pages (from-to)341-349
Number of pages9
JournalAmerican journal of hematology
Issue number3
StatePublished - Mar 1 2016

ASJC Scopus subject areas

  • Hematology


Dive into the research topics of 'Chronic neutrophilic leukemia 2016: Update on diagnosis, molecular genetics, prognosis, and management'. Together they form a unique fingerprint.

Cite this