Rapid transformation of atypical myeloproliferative disorder with consistent t(8;13) to B-cell acute lymphoblastic leukemia: A case report
Makaleler > Tam Makale · 2007
Yazarlar: Fahri ŞAHİN, Zeynep Sercan, Yeşim ERTAN, Serkan OCAKCI, Ertan Ay, Filiz VURAL, Erdinc Yuksel, Murat TOMBULOĞLU, Güray SAYDAM
Hematology, SCI Expanded İndekslerine Giren Dergi, Uluslararası, Wos, 2007, cilt 12, ss 489-492
DOI: 10.1080/10245330701562204
Özet
8p11 myeloproliferative syndrome (EMS; also known as the stem cell leukemia syndrome-SCLL) is a rare atypical myeloproliferative disorder associated with chromosomal abnormalities involving the 8p11 chromosomal band. Translocations associated with this syndrome result in the fusion of the fibroblast growth factor receptor 1 (FGFR 1) gene with various partners, resulting in ligand independent FGFR activity. The most commonly observed translocation of this syndrome is t(8;13), which results in the expression of a chimeric ZNF198-FGFR1 tyrosine kinase. Disease phenotype associated with this translocation has some typical features such as poor prognosis, and transformation to mainly acute leukemia and non-Hodgkin lymphoma; commonly with a T-cell phenotype in which obtaining and maintenance of remission is difficult by conventional chemotherapy. We hereby present a case diagnosed as atypical chronic myeloproliferative disease with consistent t(8;13)(p12;q12) and transformed rapidly to pre-B-cell acute lymphoblastic leukemia which is a rare clinical presentation.