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Myeloid/lymphoid neoplasms with eosinophilia and FGFR1 rearrangement t(8;13)(p11;q12): A case report and literature review.


ABSTRACT: 8p11 myeloproliferative syndrome (EMS) is a rare and aggressive hematological malignancy, characterized by myeloproliferative neoplasms, and associated with eosinophilia and T- or B-cell lineage lymphoblastic lymphoma. The pathogenesis is defined by the presence of chromosomal translocations associated with the fibroblast growth factor-1 (FGFR1) gene, located in the 8p11-12.1 chromosomal locus. At present, only ~100 cases have been reported globally. At least 15 partner genes have been identified, including the most common, the zinc finger MYM-type containing 2 (ZNF198)-FGFR1 fusion gene formed by t(8;13)(p11;q12). Different fusion genes determine the clinical manifestations and prognosis of the disease. Patients with EMS with t(8;13)(p11;q12) commonly present with lymphadeno

SUBMITTER: Guo YJ 

PROVIDER: S-EPMC11306990 | biostudies-literature | 2024 Oct

REPOSITORIES: biostudies-literature