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Primary cells in BCR/FGFR1-positive 8p11 myeloproliferative syndrome are sensitive to dovitinib, ponatinib, and dasatinib
Lund University, Sweden.
Linköpings universitet, Institutionen för klinisk och experimentell medicin. Linköpings universitet, Hälsouniversitetet. Region Östergötland, Centrum för kirurgi, ortopedi och cancervård, Hematologiska kliniken US.
Lund University, Sweden.
Central Hospital Skovde, Sweden.
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2017 (Engelska)Ingår i: European Journal of Haematology, ISSN 0902-4441, E-ISSN 1600-0609, Vol. 99, nr 5, s. 442-448Artikel i tidskrift (Refereegranskat) Published
Abstract [en]

ObjectivesTranslocations involving the fibroblast growth factor receptor 1 (FGFR1) gene are associated with the 8p11 myeloproliferative syndrome (EMS), a rare neoplasm that following a usually short chronic phase progresses into acute myeloid or lymphoid leukemia. The treatment commonly involves chemotherapy and, if possible, allogeneic stem cell transplantation which is the only therapeutic option for long-term survival. Given the aggressive course of EMS, we here evaluated tyrosine kinase inhibitors as treatment options to delay disease progression. MethodsWe described a new case of EMS and used chromosome analyses, PCR, and sequencing to investigate the underlying genetic aberrations. The sensitivity to several tyrosine kinase inhibitors was tested in vitro on the EMS cell line KG1 and on primary cells from the newly diagnosed EMS patient. ResultsA translocation involving chromosomes 8 and 22 was detected, and a BCR/FGFR1 fusion gene was confirmed and characterized by sequencing. KG1 cells and primary EMS cells displayed distinct sensitivity to dovitinib, ponatinib, and dasatinib as compared to normal bone marrow control cells. ConclusionsThese results suggest that treatment with tyrosine kinase inhibitors may be beneficial for patients with EMS during the search for a suitable stem cell donor and for those not eligible for transplantation.

Ort, förlag, år, upplaga, sidor
WILEY , 2017. Vol. 99, nr 5, s. 442-448
Nyckelord [en]
myeloproliferative syndrome; 8p11 myeloproliferative syndrome; BCR; FGFR1; tyrosine kinase inhibitors
Nationell ämneskategori
Hematologi
Identifikatorer
URN: urn:nbn:se:liu:diva-142830DOI: 10.1111/ejh.12957ISI: 000413152500009PubMedID: 28881484OAI: oai:DiVA.org:liu-142830DiVA, id: diva2:1154948
Tillgänglig från: 2017-11-06 Skapad: 2017-11-06 Senast uppdaterad: 2017-11-06

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