Problem kontroli leukocytozy u chorego na pierwotne włóknienie szpiku leczonego ruksolitynibem

Q4 Medicine Hematologia Pub Date : 2019-08-23 DOI:10.5603/HEM.2019.0006
M. Sobas, A. Czyż, Krzysztof Zduniak, M. Biedroń, T. Wróbel
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Abstract

Ruxolitynib — JAK1/JAK2 inhibitor — registrated for patients with myelofibrosis (MF) IPSS (International Prognostic Scoring System) intermediate-2 or high, with splenomegaly and general symptomatology. Moreover, ruxolitinib is used to reduce splenomegaly and general symptomatology in patients with MF who are candidates for allogeneic hematopoietic stem cells transplantation (allo- -HSCT). Anemia, thrombocytopenia and occasionally leukopenia with neutropenia are observed as a main side effects of ruxolitinib. Moreover, there are problems related to cytokine storm caused by treatment discontinuation. Here we report the patient with MF and important leucocytosis, in whom splenomegaly and general symptoms were reduced due to ruxolitinib. However, ruxolitinib in monotherapy did not reduce the leukocyte count, so the the combined therapy with ruxolitinib plus hydroxycarbamide was initiated. As the result of the treatment, reduction of number of leukocytes was observed, meanwhile the level of hemoglobin was stable and platelets count drop slightly. Thereafter, the patient proceeded to allo-HSCT. So far, there are two cases of patients treated with ruxolitinib plus hydroxycarbamid published in the literature. We think, that all cases treated in that way, should be reporteds, as we can establish the safety of this drug combination.
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Ruxolitynib - JAK1/JAK2抑制剂-注册用于骨髓纤维化(MF) IPSS(国际预后评分系统)中-2或高,脾大和一般症状的患者。此外,ruxolitinib可用于减少同种异体造血干细胞移植(allo- hsct)候选MF患者的脾肿大和一般症状。贫血,血小板减少和偶尔白细胞减少伴中性粒细胞减少被观察到为鲁索利替尼的主要副作用。此外,还存在因停药引起的细胞因子风暴相关问题。在这里,我们报告了一名患有MF和重要白细胞增多症的患者,在他的脾肿大和一般症状因鲁索利替尼而减轻。然而,鲁索利替尼单药治疗没有降低白细胞计数,因此开始鲁索利替尼加羟基脲联合治疗。治疗后白细胞减少,血红蛋白水平稳定,血小板计数略有下降。此后,患者进行了移植肝移植。迄今为止,文献中发表了2例ruxolitinib + hydroxycarbamid治疗的患者。我们认为,所有以这种方式治疗的病例都应该报告,因为我们可以确定这种药物组合的安全性。
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Hematologia
Hematologia Medicine-Oncology
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审稿时长
4 weeks
期刊介绍: Hematology is the quarterly under auspices of the Institute of Hematology and Transfusion Medicine. The journal is addressed to hematologists, oncologists and also internists. It contains the overview/review articles, case reports, essays, including reports from the scientific and educational conferences as well as test questions on hematology. Journal of the Institute of Hematology and Transfusiology.
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