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.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
MeSH terms
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Bone Marrow Transplantation*
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Chromosomes, Human, Pair 13*
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Chromosomes, Human, Pair 8*
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Humans
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Male
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Middle Aged
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Myeloproliferative Disorders / genetics*
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Myeloproliferative Disorders / therapy*
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Plant Proteins / genetics
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Receptor Protein-Tyrosine Kinases / genetics
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Receptor, Fibroblast Growth Factor, Type 1
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Receptors, Fibroblast Growth Factor / genetics
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Remission Induction
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Reverse Transcriptase Polymerase Chain Reaction
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Translocation, Genetic*
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Transplantation, Homologous
Substances
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FIM protein, Antirrhinum majus
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Plant Proteins
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Receptors, Fibroblast Growth Factor
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FGFR1 protein, human
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Receptor Protein-Tyrosine Kinases
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Receptor, Fibroblast Growth Factor, Type 1