Chronic neutrophilic leukemia (CNL) is a BCR-ABL1-negative myeloproliferative neoplasm with notably dismal survival. The current 2016 World Health Organization classification of myeloid neoplasms enables clinicians to unequivocally differentiate CNL from its comparable myelodysplastic/myeloproliferative neoplasm overlap syndromes. Additionally, the gradual emergence of next-generation sequencing has progressively expanded our evolving understanding of the molecular pathogenesis of CNL and its therapeutic potential. Hematopoietic stem-cell transplantation remains the primary therapeutic option for the effective treatment of CNL. In this comprehensive review, we highlight the contemporaneous classification, diagnostic criteria, and molecular pathogenesis of CNL. We also discuss the therapeutic implications of the heterogeneous molecular fingerprint of CNL, focusing on emerging targeted therapies, specifically inhibitors of JAK and MAPK signaling pathways.
Keywords: CSF3R; JAK-STAT; Myeloproliferative neoplasm; Ruxolitinib; SETBP1.
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