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Long-term complete haematological and molecular remission after allogeneic bone marrow transplantation in a patient with a stem cell myeloproliferative disorder associated with t(8;13)(p12;q12)
Authors:Suzan Florence  Guasch Géraldine  Terre Christine  Garcia Isabel  Bastie Jean-Noël  Maarek Odile  Ribaud Patricia  Gluckman Eliane  Daniel Marie-Thérèse  Pébusque Marie-Josèphe  Castaigne Sylvie
Institution:Department of Haematology, Centre Hospitalier de Versailles, INSERM U119, Marseille, France.
Abstract:A rare atypical myeloproliferative disorder (aMPD) associated with chromosomal translocations involving the short arm of chromosome 8, region p11-p12 has been described. In most patients, the cytogenetic abnormality is a t(8;13)(p12;q12) that fuses fibroblast growth factor receptor 1, the 8p12 key gene, to FIM/ZNF198 gene. Prognosis is poor with frequent evolution to acute myeloid leukaemia within 1 year of diagnosis. We report a new patient with aMPD with a t(8;13) translocation. Complete haematological, cytogenetic and molecular remission was demonstrated 39 months after allogeneic bone marrow transplantation. This is the first report to demonstrate a molecular remission in this disorder.
Keywords:myeloproliferative disorder  t(8  13)  allogeneic bone marrow transplantation  molecular remission
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