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Chronic neutrophilic leukemia and chronic myelomonocytic leukemia: WHO defined

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The World Health Organization (WHO) classification of myeloid disorders has provided updated parameters for the consistent diagnosis of two previously less than optimally defined chronic myeloid disorders, CNL and CMML. The classification of these disorders, which had been controversial, is now better defined and provides more clinically and biologically relevant disease definitions to enable uniform diagnosis and a framework to evaluate natural history and therapeutic interventions. CNL is now recognized as a distinct entity among the chronic myeloproliferative disorders and CMML is included within the new category of ‘myelodysplastic/myeloproliferative diseases’ (MDS/MPD). Predominant neutrophilia defines CNL whereas CMML is defined by predominant and monocytosis. In each case these defining features must be distinguished from reactive causes for the same in the absence of clear evidence of myeloid clonality (CNL and CMML) or dysplasia (CMML). The exclusion of underlying bcr/abl-driven oncogenesis is an essential component in the diagnosis of these chronic leukemic processes. The optimal therapy for both CNL and CMML remains uncertain. Current management decisions are based on small studies or extrapolated from therapeutic strategies that are effective in similar chronic, clonal myeloid disorders. Given the potential for evolution to acute leukemia or progressive refractory leucocytosis or cytopenias, allogeneic stem cell transplantation might be appropriate for younger patients. Continued reporting and investigation of specific therapeutic strategies and responses must be encouraged.

Section snippets

Chronic neutrophilic leukemia (CNL)

Chronic neutrophilic leukemia (CNL) is a rare chronic myeloproliferative disorder (CMPD) that has only recently attained formal recognition as a distinct entity within the updated the World Health Organization (WHO) classification system.1 CNL is characterized by sustained, mature neutrophilic leukocytosis with few or no circulating immature granulocytes, monocytosis or basophilia. Affected patients usually display splenomegaly, and bone marrow (BM) histology reveals granulocytic hyperplasia

Chronic myelomonocytic leukemia (CMML)

Chronic myelomonocytic leukemia (CMML), the classification of which has been controversial and disputed to date, is now better defined within a newly created WHO category of myeloid disorders.1, 2 It is a clonal disorder of the hematopoietic stem cell characterized by an absolute peripheral blood monocytosis accompanied by evidence of both effective (myeloproliferative) and ineffective (myelodysplastic) hematopoiesis. The coexistence of these latter contrasting features prompted the formation

Summary and conclusion

The World Health Organization (WHO) classification of myeloid disorders has provided updated parameters for the consistent diagnosis of two previously less-than-optimally defined chronic myeloid disorders, CNL and CMML. The current revised classification is more clinically and biologically relevant, enabling uniform diagnosis and a framework within which to evaluate natural history and specific therapeutic interventions of these disorders. CNL is now recognized as a distinct entity among the

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