Incidence and clinical characteristics of myeloproliferative neoplasms displaying a PDGFRB rearrangement

European Journal of Haematology
Maryam ArefiJesús M Hernández-Rivas

Abstract

The myeloproliferative neoplasms displaying a PDGFRB rearrangement are rare diseases derived from a haematopoietic stem cell. The goals of the study were to assess the incidence of these disorders and to define the clinical and biological characteristics as well as the response to the imatinib therapy. A total of 556 patients with myeloproliferative neoplasms were studied by means of molecular cytogenetics. The incidence of myeloproliferative neoplasms (MPN) with PDGFRB rearrangement was low (10 cases, 1.8% of all MPN). Most of the patients showed moderate anaemia (median Hb was 10.0 gr/dL; range from 7.5 to 13 g/dL), leukocytosis (median white blood cells was 21.7 × 10(9) /L with a range from 4 to 43 × 10(9) /L) and eosinophilia (median circulating eosinophils was 2.4 × 10(9) /L with a range of 1.1-5.7 × 10(9) /L) with a median of bone marrow infiltration cells displaying PDGFRB rearrangement of 55% (range, 37-85%). In three cases, a t(5;12) was observed while two patients showed rearrangements of 17q21 region. In two cases, a del(5)(q31) was observed. Most of the patients responded to standard dosage of imatinib, and the response was maintained in the time in those patients with a follow-up higher than 9 years. The incidence ...Continue Reading

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Citations

Mar 20, 2015·Internal Medicine·Naoto Takahashi
May 13, 2014·European Journal of Haematology·Maryam ArefiJuan Luis García
Jun 7, 2014·Blood·Francesco Passamonti
Sep 30, 2016·Archives of Pathology & Laboratory Medicine·Daniel F Boyer
Oct 22, 2013·São Paulo Medical Journal = Revista Paulista De Medicina·Alex Freire SandesEdgar Gil Rizzatti
Jan 19, 2018·International Journal of Laboratory Hematology·D T LynchK Foucar
Aug 16, 2015·American Journal of Clinical Pathology·Francisco VegaRoberto N Miranda
Jan 1, 2014·Terapevticheskiĭ arkhiv·A L MelikianT N Obukhova
Feb 18, 2017·The American Journal of Case Reports·Mirela AndreiJen Chin Wang
Sep 5, 2020·Journal of the National Comprehensive Cancer Network : JNCCN·Aaron T GerdsHema Sundar
Jan 5, 2021·Current Opinion in Pediatrics·Yana Pikman, Elliot Stieglitz

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