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

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 diagn...

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Main Authors: Panagiotis T. Diamantopoulos, Nora-Athina Viniou
Format: Article
Language:English
Published: Frontiers Media S.A. 2021-11-01
Series:Frontiers in Oncology
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fonc.2021.722507/full
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author Panagiotis T. Diamantopoulos
Nora-Athina Viniou
author_facet Panagiotis T. Diamantopoulos
Nora-Athina Viniou
author_sort Panagiotis T. Diamantopoulos
collection DOAJ
description 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.
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spelling doaj.art-738dd1804ff64cda93ea9196008307a22022-12-21T23:14:15ZengFrontiers Media S.A.Frontiers in Oncology2234-943X2021-11-011110.3389/fonc.2021.722507722507Atypical Chronic Myelogenous Leukemia, BCR-ABL1 Negative: Diagnostic Criteria and Treatment ApproachesPanagiotis T. DiamantopoulosNora-Athina ViniouAtypical 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.https://www.frontiersin.org/articles/10.3389/fonc.2021.722507/fullatypical chronic myelogenous leukemiamyelodysplastic syndrome/myeloproliferative neoplasmdiagnostic criteriatreatmentmolecular characterization
spellingShingle Panagiotis T. Diamantopoulos
Nora-Athina Viniou
Atypical Chronic Myelogenous Leukemia, BCR-ABL1 Negative: Diagnostic Criteria and Treatment Approaches
Frontiers in Oncology
atypical chronic myelogenous leukemia
myelodysplastic syndrome/myeloproliferative neoplasm
diagnostic criteria
treatment
molecular characterization
title Atypical Chronic Myelogenous Leukemia, BCR-ABL1 Negative: Diagnostic Criteria and Treatment Approaches
title_full Atypical Chronic Myelogenous Leukemia, BCR-ABL1 Negative: Diagnostic Criteria and Treatment Approaches
title_fullStr Atypical Chronic Myelogenous Leukemia, BCR-ABL1 Negative: Diagnostic Criteria and Treatment Approaches
title_full_unstemmed Atypical Chronic Myelogenous Leukemia, BCR-ABL1 Negative: Diagnostic Criteria and Treatment Approaches
title_short Atypical Chronic Myelogenous Leukemia, BCR-ABL1 Negative: Diagnostic Criteria and Treatment Approaches
title_sort atypical chronic myelogenous leukemia bcr abl1 negative diagnostic criteria and treatment approaches
topic atypical chronic myelogenous leukemia
myelodysplastic syndrome/myeloproliferative neoplasm
diagnostic criteria
treatment
molecular characterization
url https://www.frontiersin.org/articles/10.3389/fonc.2021.722507/full
work_keys_str_mv AT panagiotistdiamantopoulos atypicalchronicmyelogenousleukemiabcrabl1negativediagnosticcriteriaandtreatmentapproaches
AT noraathinaviniou atypicalchronicmyelogenousleukemiabcrabl1negativediagnosticcriteriaandtreatmentapproaches