Efficacy and safety of ruxolitinib in regularly transfused patients with thalassemia: results from a phase 2a study.
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SUBMITTER: Taher AT
PROVIDER: S-EPMC5757682 | biostudies-literature | 2018 Jan
REPOSITORIES: biostudies-literature
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Efficacy and safety of ruxolitinib in regularly transfused patients with thalassemia: results from a phase 2a study.
Taher Ali T AT Karakas Zeynep Z Cassinerio Elena E Siritanaratkul Noppadol N Kattamis Antonis A Maggio Aurelio A Rivella Stefano S Hollaender Norbert N Mahuzier Bruyère B Gadbaw Brian B Aydinok Yesim Y
Blood 20171102 2
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Project description:Chronically transfused patients with thalassemia are at risk for red cell alloimmunization. No studies have specifically examined alloimmunization after implementation of prophylactic Rh (D, C, E) and K matched red cells in a racially diverse population of thalassemia patients and donors. This retrospective study examined Rh antibodies among 40 chronically transfused patients (Asian, White, Black, Indian, Middle Eastern) with thalassemia receiving a mean of 174 serologic prophylactic RhD, C, E, and K matched red cell units. We examined the patients' RH genotype, as well as donor race and Rh phenotypes over 3 transfusion events preceding antibody detection. Eighteen alloantibodies were detected in 13 of 40 patients (32.5%), with an alloimmunization rate of 0.26 antibodies per 100 units transfused. Thirteen antibodies (72.2%) were directed against Rh (5 anti-D, 4 anti-C, 2 anti-E, 1 anti-e, 1 anti-V), despite donor phenotypes that confirmed lack of transfusion of D, C, or E antigens to patients lacking the corresponding antigen(s). Ten of 40 patients had an altered RH genotype, but the Rh antibodies were not associated with patients with variant RH. Black donors with a known high frequency of RH variants provided 63% of the units transfused in the 3 visits preceding unexplained anti-Rh detection. Rh alloimmunization not explained by the thalassemia patients' RH genotype or the donors' serologic phenotype suggests more precise matching is needed, and the role of donor RH genotypes on alloimmunization should be explored. Extending Rh D, C, and E matching to include c and e would result in better-matched units and further minimize Rh alloimmunization.
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