Update on CML-Like Disorders |
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| Affiliation: | 1. University of Southampton, Southampton, UK;2. Wessex Regional Genetics Laboratory, Salisbury NHS Foundation Trust, Salisbury, UK;1. Department of Hematology, Niguarda Cancer Center, Niguarda Hospital, Milan, Italy;1. Department of Leukemia, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, Texas, 77030, United States;1. Division of Oncology, Washington University School of Medicine, St Louis, MO, USA;1. Institut de Cancérologie Gustave Roussy, Villejuif, France;1. Massachusetts General Hospital, Boston, MA;1. Roswell Park Comprehensive Cancer Center, Elm and Carlton Streets, Buffalo, NY, 14263, USA |
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| Abstract: | Chronic myeloid leukemia (CML) is defined for many years as BCR-ABL1 positive disease, but older publications refer to a poor prognosis, clinically heterogeneous entity termed ‘BCR-ABL1 negative CML’ constituting about 5% of CML cases. Apart from very rare CML cases with cytogenetically cryptic, atypical variant BCR-ABL1 fusions that had been inadvertently missed during the diagnostic work up, most of these cases would now be classified as a subtype of myelodysplastic/myeloproliferative neoplasm (MDS/MPN), such as atypical CML (aCML), chronic myelomonocytic leukemia (CMML), or chronic neutrophilic leukemia (CNL). A minority would be classified as systemic mastocytosis with associated hematological neoplasm (SM-AHN), myeloid/lymphoid neoplasms associated with eosinophilia and rearrangement of PDGFRA, PDGFRB, FGFR1 or with PCM1-JAK2 (MLN-eo), or chronic eosinophilic leukemia not otherwise specified (CEL-NOS).1 |
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| Keywords: | Tyrosine kinase fusions eosinophilia atypical CML targeted therapy |
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