436
edits
Changes
→Cancer Category/Type
The clinical/laboratory characteristics typically reflect features of chronic myeloid neoplasms and variable eosinophilia.9 Patients may also present as de novo AML without an antecedent MPN. There is a high incidence of T-lymphoblastic lymphomas, particularly in association with a t(8;13) and a ZMYM2-FGFR1 fusion gene,9,53,54 which may occur at diagnosis or during the course of disease, reflecting a myeloid/lymphoid stem cell origin. The clinical course is aggressive as a result of rapid progression to blast phase/secondary acute leukemia, usually of myeloid phenotype, less commonly B-ALL, within 1 or 2 years of diagnosis. The variability in the clinical presentation may be a result of specific moieties of the partner genes and signaling via different intracellular pathways.59 The t(8;22) is often associated with a clinical and hematologic picture very similar to that seen in BCR-ABL1-positive CML with basophilia,58,60 whereas thrombocytopenia and monocytosis resembling CMML are more frequently present in t(6;8)56 and t(8;9).55,61 The t(6;8) may also present with a PV-like disease,62 and eosinophilia may be absent in t(6;8) and t(8;22).(Reiter and Gotleib)
The clinical/laboratory characteristics typically reflect features of chronic myeloid neoplasms and variable eosinophilia.9 Patients may also present as de novo AML without an antecedent MPN. There is a high incidence of T-lymphoblastic lymphomas, particularly in association with a t(8;13) and a ZMYM2-FGFR1 fusion gene,9,53,54 which may occur at diagnosis or during the course of disease, reflecting a myeloid/lymphoid stem cell origin. The clinical course is aggressive as a result of rapid progression to blast phase/secondary acute leukemia, usually of myeloid phenotype, less commonly B-ALL, within 1 or 2 years of diagnosis. The variability in the clinical presentation may be a result of specific moieties of the partner genes and signaling via different intracellular pathways.59 The t(8;22) is often associated with a clinical and hematologic picture very similar to that seen in BCR-ABL1-positive CML with basophilia,58,60 whereas thrombocytopenia and monocytosis resembling CMML are more frequently present in t(6;8)56 and t(8;9).55,61 The t(6;8) may also present with a PV-like disease,62 and eosinophilia may be absent in t(6;8) and t(8;22).(Reiter and Gotleib)
In a recent report (Wehrli et al, 2017) a patient diagnosed with EMS and a CEP110-FGFR1 rearrangement responded to treatment with the tyrosine kinase inhibitor (TKI) dasatinib.
==Gene Overview==
==Gene Overview==