8p11 Myeloproliferative Syndrome with t(7;8) Translocation Presenting as Acute Myeloid Leukemia: A Case Report and Literature Review

Byun, Ja Min; Lee, Jeong-Ok; Bang, Soo Mee; Yu, Eun-Jeong; Hwang, Sang Mee; Lee, Dongsoon

doi:10.1159/000444627

Cited by 2 publications

(1 citation statement)

References 10 publications

(12 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…There are 5 known cases, three of which were found in Korea. One of the patients was not accompanied by eosinophilia at the time of onset and had increased but it subsided spontaneously ( 82 ). After nearly 5 years, eosinophilia appeared in the patient’s peripheral blood and bone marrow, and the clinical diagnosis was chronic eosinophilic leukemia.…”

Section: Genotypic and Phenotypic Classificationmentioning

confidence: 99%

The 8p11 myeloproliferative syndrome: Genotypic and phenotypic classification and targeted therapy

Zhang

Lin

et al. 2022

Front. Oncol.

View full text Add to dashboard Cite

EMS(8p11 myeloproliferative syndrome, EMS) is an aggressive hematological neoplasm with/without eosinophilia caused by a rearrangement of the FGFR1 gene at 8p11-12. It was found that all cases carry chromosome abnormalities at the molecular level, not only the previously reported chromosome translocation and insertion but also a chromosome inversion. These abnormalities produced 17 FGFR1 fusion genes, of which the most common partner genes are ZNF198 on 13q11-12 and BCR of 22q11.2. The clinical manifestations can develop into AML (acute myeloid leukemia), T-LBL (T-cell lymphoblastic lymphoma), CML (chronic myeloid leukemia), CMML (chronic monomyelocytic leukemia), or mixed phenotype acute leukemia (MPAL). Most patients are resistant to traditional chemotherapy, and a minority of patients achieve long-term clinical remission after stem cell transplantation. Recently, the therapeutic effect of targeted tyrosine kinase inhibitors (such as pemigatinib and infigratinib) in 8p11 has been confirmed in vitro and clinical trials. The TKIs may become an 8p11 treatment option as an alternative to hematopoietic stem cell transplantation, which is worthy of further study.

show abstract

Section: Genotypic and Phenotypic Classificationmentioning

confidence: 99%