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Diagnostic Criteria of Atypical Chronic Myeloid Leukemia (aCML)

Atypical chronic myelogenous leukemia (aCML), BCR/ABL1 negative is a rare disorder classified into the category of myelodysplastic/myeloproliferative neoplasms (MDS/MPN), according to the 2016 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia. It is, by definition, a BCR-ABL1-negative clonal disorder sharing myelodysplastic and myeloproliferative features. Continue reading “Diagnostic Criteria of Atypical Chronic Myeloid Leukemia (aCML)”

Diagnostic Criteria for Chronic Neutrophilic Leukemia (CNL)

Chronic neutrophilic leukemia (CNL) is a rare, often aggressive myeloproliferative neoplasm (MPN) defined by persistent mature neutrophilic leukocytosis, bone marrow granulocyte hyperplasia, and frequent hepatosplenomegaly. The seminal discovery of oncogenic driver mutations in colony-stimulating factor 3 receptor (CSF3R) in the majority of patients with CNL in 2013 anchored a new scientific framework, deepening our understanding of its molecular pathogenesis, providing a diagnostic biomarker, and rationalizing the use of pharmacological targeting. Continue reading “Diagnostic Criteria for Chronic Neutrophilic Leukemia (CNL)”

Accelerated Phase Criteria for Chronic Myeloid Leukemia (CML)

The chronic myeloid leukemia (CML) is classically staged into chronic phase (CP, most patients at presentation), accelerated phase (AP) and blast phase (BP). Many definitions have been used for these stages, but all the data generated from the tyrosine kinase inhibitor (TKI) studies has used the historically standard definition where AP is defined by the presence of one or more of the following: ≥15% blasts in PB/BM, ≥20% basophils in PB, platelets <100,000/µL unrelated to treatment or the development of cytogenetic evolution. Blast phase is defined by the presence of ≥30% blasts in the peripheral blood or bone marrow, the presence of clusters of blasts in marrow or the presence of extramedullary disease with immature cells (i.e., a myeloid sarcoma). Continue reading “Accelerated Phase Criteria for Chronic Myeloid Leukemia (CML)”

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