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).

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.

• Authors: Florence Suzan, Géraldine Guasch, Christine Terre, Isabel Garcia, Jean-Noël Bastie, Odile Maarek, Patricia Ribaud, Eliane Gluckman, Marie-Thérèse Daniel, Marie-Josèphe Pébusque, Sylvie Castaigne
• Journal: British journal of haematology (Br J Haematol) Vol. 121 Issue 2 Pg. 312-4 (Apr 2003) ISSN: 0007-1048 [Print] England
• PMID: 12694254 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• FIM protein, Antirrhinum majus
• Plant Proteins
• Receptors, Fibroblast Growth Factor
• FGFR1 protein, human
• Receptor Protein-Tyrosine Kinases
• Receptor, Fibroblast Growth Factor, Type 1

Topics

• Bone Marrow Transplantation
• Chromosomes, Human, Pair 13
• Chromosomes, Human, Pair 8
• Humans
• Male
• Middle Aged
• Myeloproliferative Disorders (genetics, therapy)
• Plant Proteins (genetics)
• Receptor Protein-Tyrosine Kinases (genetics)
• Receptor, Fibroblast Growth Factor, Type 1
• Receptors, Fibroblast Growth Factor (genetics)
• Remission Induction
• Reverse Transcriptase Polymerase Chain Reaction
• Translocation, Genetic
• Transplantation, Homologous