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Review
. 2021 Nov 17:11:722507.
doi: 10.3389/fonc.2021.722507. eCollection 2021.

Atypical Chronic Myelogenous Leukemia, BCR-ABL1 Negative: Diagnostic Criteria and Treatment Approaches

Panagiotis T Diamantopoulos  1 , Nora-Athina Viniou  1
Affiliations
Review

Atypical Chronic Myelogenous Leukemia, BCR-ABL1 Negative: Diagnostic Criteria and Treatment Approaches

Panagiotis T Diamantopoulos et al. Front Oncol. .

Abstract

Atypical chronic myelogenous leukemia (aCML), BCR/ABL1 negative is a rare myelodysplastic/myeloproliferative neoplasm, usually manifested with hyperleukocytosis without monocytosis or basophilia, organomegaly, and marked dysgranulopoiesis. In this review, we will discuss the classification and diagnostic criteria of aCML, as these have been formulated during the past 30 years, with a focus on the recent advances in the molecular characterization of the disease. Although this entity does not have a definitive molecular profile, its molecular characterization has contributed to a better understanding and more accurate classification and diagnosis of aCML. At the same time, it has facilitated the identification of adverse prognostic factors and the stratification of patients according to their risk for leukemic transformation. What is more, the molecular characterization of the disease has expanded our therapeutic choices, thoroughly presented and analyzed in this review article.

Keywords: atypical chronic myelogenous leukemia; diagnostic criteria; molecular characterization; myelodysplastic syndrome/myeloproliferative neoplasm; treatment.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Proposed algorithm for the diagnosis of aCML. MDS/MPN, myelodysplastic syndrome/myeloproliferative neoplasm; CML, chronic myeloid leukemia; CMML, chronic myelomonocytic leukemia; FISH, fluorescent in situ hybridization; RS, ring sideroblasts; T, thrombocytosis; CNL, chronic neutrophilic leukemia; aCML, atypical chronic myelogenous leukemia, BCR/ABL1 negative.
See this image and copyright information in PMC

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