成纤维细胞生长因子受体1
骨髓增生性疾病
癌症研究
慢性粒单核细胞白血病
染色体易位
成纤维细胞生长因子受体
医学
白血病
造血
酪氨酸激酶
急性粒单核细胞白血病
融合基因
急性白血病
内科学
作者
Marie-Joelle Mozziconacci,Nadine Carbuccia,Thomas Prebet,Aude Charbonnier,Anne Murati,Norbert Vey,Max Chaffanet,Daniel Birnbaum
标识
DOI:10.1016/j.leukres.2007.11.012
摘要
The 8p12 myeloproliferative syndrome is a rare, generally aggressive chronic myeloproliferative disorder (MPD). The hallmark of this MPD is the disruption of the FGFR1 gene, which encodes a tyrosine kinase receptor for members of the fibroblast growth factor family. In MPD cells FGFR1 is fused to several partners. The most frequent partner genes are BCR, CEP110, FOP, and ZNF198, localized on 22q11, 9q33, 6q27, and 13q12, respectively. We report here the tenth case of translocation (8;9)(p12;q33) in an acute myelomonocytic leukemia and provide a review of the literature that points to common syndrome features: the t(8;9)(p11;q33) MPD transforms rapidly, and always in myelomonocytic leukemia, with a possible B- or T-lymphoid involvement, which may include tonsil invasion. The FGFR1-MPD seems refractory to current chemotherapies and is not sensitive to imatinib. Currently, only the patients with bone marrow transplantation stand a chance of survival.
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