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)

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 g...

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Veröffentlicht in:British journal of haematology 2003-04, Vol.121 (2), p.312-314
Hauptverfasser: 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
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Sprache:eng
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Zusammenfassung: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.
ISSN:0007-1048
1365-2141
DOI:10.1046/j.1365-2141.2003.04269.x