Chronic myelomonocytic leukemia as a transformation from polycythemia vera

Andrei, Mirela; Pandey, Anita; Avezbakiyev, Boris; Wang, Jen C.

doi:10.1016/j.lrr.2019.03.004

Cited by 4 publications

(3 citation statements)

References 16 publications

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“…Of course, we cannot generalize this observation to all CMML-developing MPN patients. Indeed, there was a case report showing CMML as a transformation from PV and demonstrating that the CMML clone is most likely derived from the PV-JAK2 clone [ 23 ]. Moreover, in JAK2 -mutated MPNs two routes of leukemic transformation have been demonstrated by analyzing 16 patients with a JAK2 -mutant or JAK2 wild-type acute myeloid leukemia after a JAK2 -mutated MPN [ 24 ].…”

Section: Discussionmentioning

confidence: 99%

Clinical, Hematologic, Biologic and Molecular Characteristics of Patients with Myeloproliferative Neoplasms and a Chronic Myelomonocytic Leukemia-Like Phenotype

Heibl

Gisslinger

Jäger

et al. 2020

Cancers

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Patients with a myeloproliferative neoplasm (MPN) sometimes show a chronic myelomonocytic leukemia (CMML)-like phenotype but, according to the 2016 WHO classification, a documented history of an MPN excludes the diagnosis of CMML. Forty-one patients with an MPN (35 polycythemia vera (PV), 5 primary myelofibrosis, 1 essential thrombocythemia) and a CMML-like phenotype (MPN/CMML) were comprehensively characterized regarding clinical, hematologic, biologic and molecular features. The white blood cell counts in MPN/CMML patients were not different from CMML patients and PV patients. The hemoglobin values and platelet counts of these patients were higher than in CMML but lower than in PV, respectively. MPN/CMML patients showed myelomonocytic skewing, a typical in vitro feature of CMML but not of PV. The mutational landscape of MPN/CMML was not different from JAK2-mutated CMML. In two MPN/CMML patients, development of a CMML-like phenotype was associated with a decrease in the JAK2 V617F allelic burden. Finally, the prognosis of MPN/CMML (median overall survival (OS) 27 months) was more similar to CMML (JAK2-mutated, 28 months; JAK2-nonmutated 29 months) than to PV (186 months). In conclusion, we show that patients with MPN and a CMML-like phenotype share more characteristics with CMML than with PV, which may be relevant for their classification and clinical management.

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Section: Discussionmentioning

confidence: 99%

Clinical, Hematologic, Biologic and Molecular Characteristics of Patients with Myeloproliferative Neoplasms and a Chronic Myelomonocytic Leukemia-Like Phenotype

Heibl

Gisslinger

Jäger

et al. 2020

Cancers

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“…In an article published by Holcombe in 1991, 2 cases similar to the case described herein were reported, but genetic analyses were not available in those patients 9 . Recently, Andrei et al published a case report describing a CMML transformation from PV with acquired mutations other than JAK2 10 . In this case they confirmed that the CD14 + population carried the JAK2 V617F mutation, and they concluded that the CMML was originated from the PV clone.…”

Section: Figurementioning

confidence: 67%

“…In this case they confirmed that the CD14 + population carried the JAK2 V617F mutation, and they concluded that the CMML was originated from the PV clone. On the other hand, the JAK2 V617F mutation is reported to be present in 7.8% of de novo CMML cases, 10 especially in proliferative subtypes.…”

Section: Figurementioning

confidence: 99%

Polycythemia Vera Evolution to Chronic Myelomocytic Leukemia: The Prognostic Value of Next Generation Sequencing

et al. 2020

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variants: Class 1: relevant in the clinical management of myeloid hemopathies. It has been established as a pathogenic variant in myeloid hemopathies and alters an actionable gene. Class 2: it has been established as a pathogenic variant in solid tumors or non-myeloid hemopathies and alters an actionable gene. Class 3: variant not previously described, affects an actionable gene and in silico predictors or classifies mutations as:• Class 3A: likely pathogenic.• Class 3B: uncertain significance (VUS).• Class 3C: likely benign.† VAF: variant allele frequency.‡ This variant has not been previously described in public databases (either as somatic or germline) and in silico predictors classified it, some as benign and others as pathogenic. In this situation, NGS recommendations suggest to classify these variants as of uncertain significance. Chr = chromosome.

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2021

Flow Cytometry of Hematological Malignancies

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Chronic myelomonocytic leukemia as a transformation from polycythemia vera

Cited by 4 publications

References 16 publications

Clinical, Hematologic, Biologic and Molecular Characteristics of Patients with Myeloproliferative Neoplasms and a Chronic Myelomonocytic Leukemia-Like Phenotype

Clinical, Hematologic, Biologic and Molecular Characteristics of Patients with Myeloproliferative Neoplasms and a Chronic Myelomonocytic Leukemia-Like Phenotype

Polycythemia Vera Evolution to Chronic Myelomocytic Leukemia: The Prognostic Value of Next Generation Sequencing

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