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2015 ; 8
(ä): 2383-90
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Chronic neutrophilic leukemia: a clinical perspective
#MMPMID26366092
Menezes J
; Cigudosa JC
Onco Targets Ther
2015[]; 8
(ä): 2383-90
PMID26366092
show ga
Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative neoplasm (MPN)
that includes only 150 patients described to date meeting the latest World Health
Organization (WHO) criteria and the recently reported CSF3R mutations. The
diagnosis is based on morphological criteria of granulocytic cells and the
exclusion of genetic drivers that are known to occur in others MPNs, such as
BCR-ABL1, PDGFRA/B, or FGFR1 rearrangements. However, this scenario changed with
the identification of oncogenic mutations in the CSF3R gene in approximately 83%
of WHO-defined and no monoclonal gammopathy-associated CNL patients. CSF3R T618I
is a highly specific molecular marker for CNL that is sensitive to inhibition in
vitro and in vivo by currently approved protein kinase inhibitors. In addition to
CSF3R mutations, other genetic alterations have been found, notably mutations in
SETBP1, which may be used as prognostic markers to guide therapeutic decisions.
These findings will help to understand the pathogenesis of CNL and greatly impact
the clinical management of this disease. In this review, we discuss the new
genetic alterations recently found in CNL and the clinical perspectives in its
diagnosis and treatment. Fortunately, since the diagnosis of CNL is not based on
exclusion anymore, the molecular characterization of the CSF3R gene must be
included in the WHO criteria for CNL diagnosis.
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