Myelodysplastic syndrome/myeloproliferative neoplasm overlap syndromes: a focused review

Myelodysplastic syndrome (MDS)/myeloproliferative neoplasm (MPN) overlap syndromes are unique myeloid neoplasms, with overlapping features of MDS and MPN. They consist of four adult onset entities including chronic myelomonocytic leukemia (CMML), MDS/MPN-ring sideroblasts-thrombocytosis (MDS/MPN-RS-...

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Bibliographic Details
Published in:Hematology 2020-12, Vol.2020 (1), p.460-464
Main Authors: Patnaik, Mrinal M, Lasho, Terra
Format: Article
Language:English
Subjects:
Administration, Oral
Antineoplastic Agents - therapeutic use
Humans
Leukemia, Myelogenous, Chronic, BCR-ABL Positive - drug therapy
Leukemia, Myelogenous, Chronic, BCR-ABL Positive - genetics
Leukemia, Myelogenous, Chronic, BCR-ABL Positive - metabolism
Leukemia, Myelogenous, Chronic, BCR-ABL Positive - pathology
Leukemia, Myelomonocytic, Juvenile - drug therapy
Leukemia, Myelomonocytic, Juvenile - genetics
Leukemia, Myelomonocytic, Juvenile - metabolism
Leukemia, Myelomonocytic, Juvenile - pathology
Mutation
Myelodysplastic Syndromes - drug therapy
Myelodysplastic Syndromes - genetics
Myelodysplastic Syndromes - metabolism
Myelodysplastic Syndromes - pathology
Myeloproliferative Disorders: Too Many Cells, Too Few Therapies
Neoplasm Proteins - genetics
Neoplasm Proteins - metabolism
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Summary:Myelodysplastic syndrome (MDS)/myeloproliferative neoplasm (MPN) overlap syndromes are unique myeloid neoplasms, with overlapping features of MDS and MPN. They consist of four adult onset entities including chronic myelomonocytic leukemia (CMML), MDS/MPN-ring sideroblasts-thrombocytosis (MDS/MPN-RS-T), BCR-ABL1 negative atypical chronic myeloid leukemia (aCML) and MDS/MPN-unclassifiable (MDS/MPN-U); with juvenile myelomonocytic leukemia (JMML) being the only pediatric onset entity. Among these overlap neoplasms, CMML is the most frequent and is hallmarked by the presence of sustained peripheral blood monocytosis with recurrent mutations involving TET2 (60%), SRSF2 (50%) and ASXL1 (40%); with RAS pathway mutations and JAK2V617F being relatively enriched in proliferative CMML subtypes (WBC ≥13 × 109/L). CMML usually presents in the 7th decade of life, with a male preponderance and is associated with a median overall survival of
ISSN:1520-4383
1520-4391
1520-4383
DOI:10.1182/HEMATOLOGY.2020000163