t(8;22)/BCR-FGFR1 myeloproliferative disorder presenting as B-acute lymphoblastic leukemia: Report of a case treated with sorafenib and review of the literature2011-09-01 18:23:54
Leukemia Research; 2011 Sept; 35(9):e151-53
MR Conces, SL Cook, EB Stelow, RD LeGallo
The 8p11 myeloproliferative syndrome (EMS) is a rare and aggressive hematological neoplasm caused by rearrangements involving fibroblast growth factor receptor 1 (FGFR1) gene on chromosome 8p11, and one of 11 identified partner genes. The result is a variety of fusion genes encoding aberrant tyrosine kinases and activating multiple signal transduction pathways. Involvement of t(8;22)/BCR-FGFR1 is exceedingly rare, with only 8 cases reported to date. It usually presents as chronic myelogenous leukemia (CML)-like disease rapidly evolving into acute myeloid leukemia (AML), but one reported case presented as Bacute lymphoblastic leukemia (B-ALL). Herein, we report the second case of t(8;22) presenting as B-ALL, and the first to be treated with targeted therapy against tyrosine kinase following chemotherapy.
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