Chronic neutrophilic leukemia

New science and new diagnostic criteria

Natasha Szuber, Ayalew Tefferi

Research output: Contribution to journalReview article

3 Citations (Scopus)

Abstract

Chronic neutrophilic leukemia (CNL) is a distinct myeloproliferative neoplasm defined by persistent, predominantly mature neutrophil proliferation, marrow granulocyte hyperplasia, and frequent splenomegaly. The seminal discovery of oncogenic driver mutations in CSF3R in the majority of patients with CNL in 2013 generated a new scientific framework for this disease as it deepened our understanding of its molecular pathogenesis, provided a biomarker for diagnosis, and rationalized management using novel targeted therapies. Consequently, in 2016, the World Health Organization (WHO) revised the diagnostic criteria for CNL to reflect such changes in its genomic landscape, now including the presence of disease-defining activating CSF3R mutations as a key diagnostic component of CNL. In this communication, we provide a background on the history of CNL, its clinical and hemopathologic features, and its molecular anatomy, including relevant additional genetic lesions and their significance. We also outline the recently updated WHO diagnostic criteria for CNL. Further, the natural history of the disease is reviewed as well as potential prognostic variables. Finally, we summarize and discuss current treatment options as well as prospective novel therapeutic targets in hopes that they will yield meaningful improvements in patient management and outcomes.

Original languageEnglish (US)
Article number19
JournalBlood Cancer Journal
Volume8
Issue number2
DOIs
StatePublished - Feb 1 2018

Fingerprint

Leukemia, Neutrophilic, Chronic
Mutation
Splenomegaly
Granulocytes
Hyperplasia
Anatomy
Neutrophils
Therapeutics
Biomarkers
Bone Marrow
History

ASJC Scopus subject areas

  • Hematology
  • Oncology

Cite this

Chronic neutrophilic leukemia : New science and new diagnostic criteria. / Szuber, Natasha; Tefferi, Ayalew.

In: Blood Cancer Journal, Vol. 8, No. 2, 19, 01.02.2018.

Research output: Contribution to journalReview article

@article{ba12e8e697ca4ebabf69f74016d8e926,
title = "Chronic neutrophilic leukemia: New science and new diagnostic criteria",
abstract = "Chronic neutrophilic leukemia (CNL) is a distinct myeloproliferative neoplasm defined by persistent, predominantly mature neutrophil proliferation, marrow granulocyte hyperplasia, and frequent splenomegaly. The seminal discovery of oncogenic driver mutations in CSF3R in the majority of patients with CNL in 2013 generated a new scientific framework for this disease as it deepened our understanding of its molecular pathogenesis, provided a biomarker for diagnosis, and rationalized management using novel targeted therapies. Consequently, in 2016, the World Health Organization (WHO) revised the diagnostic criteria for CNL to reflect such changes in its genomic landscape, now including the presence of disease-defining activating CSF3R mutations as a key diagnostic component of CNL. In this communication, we provide a background on the history of CNL, its clinical and hemopathologic features, and its molecular anatomy, including relevant additional genetic lesions and their significance. We also outline the recently updated WHO diagnostic criteria for CNL. Further, the natural history of the disease is reviewed as well as potential prognostic variables. Finally, we summarize and discuss current treatment options as well as prospective novel therapeutic targets in hopes that they will yield meaningful improvements in patient management and outcomes.",
author = "Natasha Szuber and Ayalew Tefferi",
year = "2018",
month = "2",
day = "1",
doi = "10.1038/s41408-018-0049-8",
language = "English (US)",
volume = "8",
journal = "Blood Cancer Journal",
issn = "2044-5385",
publisher = "Nature Publishing Group",
number = "2",

}

TY - JOUR

T1 - Chronic neutrophilic leukemia

T2 - New science and new diagnostic criteria

AU - Szuber, Natasha

AU - Tefferi, Ayalew

PY - 2018/2/1

Y1 - 2018/2/1

N2 - Chronic neutrophilic leukemia (CNL) is a distinct myeloproliferative neoplasm defined by persistent, predominantly mature neutrophil proliferation, marrow granulocyte hyperplasia, and frequent splenomegaly. The seminal discovery of oncogenic driver mutations in CSF3R in the majority of patients with CNL in 2013 generated a new scientific framework for this disease as it deepened our understanding of its molecular pathogenesis, provided a biomarker for diagnosis, and rationalized management using novel targeted therapies. Consequently, in 2016, the World Health Organization (WHO) revised the diagnostic criteria for CNL to reflect such changes in its genomic landscape, now including the presence of disease-defining activating CSF3R mutations as a key diagnostic component of CNL. In this communication, we provide a background on the history of CNL, its clinical and hemopathologic features, and its molecular anatomy, including relevant additional genetic lesions and their significance. We also outline the recently updated WHO diagnostic criteria for CNL. Further, the natural history of the disease is reviewed as well as potential prognostic variables. Finally, we summarize and discuss current treatment options as well as prospective novel therapeutic targets in hopes that they will yield meaningful improvements in patient management and outcomes.

AB - Chronic neutrophilic leukemia (CNL) is a distinct myeloproliferative neoplasm defined by persistent, predominantly mature neutrophil proliferation, marrow granulocyte hyperplasia, and frequent splenomegaly. The seminal discovery of oncogenic driver mutations in CSF3R in the majority of patients with CNL in 2013 generated a new scientific framework for this disease as it deepened our understanding of its molecular pathogenesis, provided a biomarker for diagnosis, and rationalized management using novel targeted therapies. Consequently, in 2016, the World Health Organization (WHO) revised the diagnostic criteria for CNL to reflect such changes in its genomic landscape, now including the presence of disease-defining activating CSF3R mutations as a key diagnostic component of CNL. In this communication, we provide a background on the history of CNL, its clinical and hemopathologic features, and its molecular anatomy, including relevant additional genetic lesions and their significance. We also outline the recently updated WHO diagnostic criteria for CNL. Further, the natural history of the disease is reviewed as well as potential prognostic variables. Finally, we summarize and discuss current treatment options as well as prospective novel therapeutic targets in hopes that they will yield meaningful improvements in patient management and outcomes.

UR - http://www.scopus.com/inward/record.url?scp=85042021394&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85042021394&partnerID=8YFLogxK

U2 - 10.1038/s41408-018-0049-8

DO - 10.1038/s41408-018-0049-8

M3 - Review article

VL - 8

JO - Blood Cancer Journal

JF - Blood Cancer Journal

SN - 2044-5385

IS - 2

M1 - 19

ER -