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Myeloproliferative disorders with t(8;9)(p12;q33): a case report and review of the literature.

Authors
Type
Published Article
Journal
Pediatric Hematology and Oncology
1521-0669
Publisher
Informa UK (Taylor & Francis)
Publication Date
Volume
28
Issue
2
Pages
140–146
Identifiers
DOI: 10.3109/08880018.2010.528170
PMID: 21214407
Source
Medline
License
Unknown

Abstract

The 8p11 myeloproliferative syndrome (EMS) is an aggressive neoplasm associated with chromosomal translocations involving the fibroblast growth factor receptor 1 tyrosine kinase gene on chromosome 8p11-12. The most frequent partner genes are in decreasing order of frequency: ZNF198 (or ZMYM2, zinc finger MYM type 2), CEP110 (centrosomal protein 110 kDa), FOP (or FGFR1OP, FGFR1 [fibroblast growth factor receptor 1] oncogene partner), and BCR (breakpoint cluster region) located on 13q12, 9q33, 6q27, and 22q11, respectively. Here the authors report a new case of translocation (8;9)(p12;q33) without lymphoma prior to the progression into acute leukemia. Currently, only patients underwent bone marrow transplantation stand a chance of long-term survival. In the future, FGFR1 inhibitor might be the specific and effective therapeutic target for EMS.

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