来那度胺治疗骨髓增生异常/骨髓增生性肿瘤的疗效,不可分类(MDS/MPN-U),存在5q -缺失和8+三体

Oliwia Bachanek-Mitura, A. Szudy-Szczyrek, M. Hus
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引用次数: 0

摘要

骨髓增生异常/骨髓增生性肿瘤(MDS/MPN)是一种非常罕见的克隆性骨髓性肿瘤,其特征是同时存在红细胞和/或粒细胞增生异常,并伴有骨髓增生性特征。这种疾病的病因尚不清楚。MDS/MPN组由五种疾病组成:慢性髓细胞白血病、非典型慢性髓细胞白血病、少年髓细胞白血病、伴有血小板增多的MDS/MPN环状铁母细胞和MDS/MPN,不可分类(MDS/MPN- u)。MDS/MPN-U仍然是最不具特征的实体。它占所有髓系疾病的不到5%。目前尚无明确的诊断标准。这种疾病具有异质性,管理策略非常明确。本文的目的是报道一例59岁女性MDS/MPN-U伴5q -缺失和8+三体,每日10mg来那度胺作为单药治疗。该病最初表现为严重的细胞减少,需要多次输血。目前患者处于治疗的第9个月。血红蛋白、血小板和白细胞恢复正常。病人完全不需要输血浓缩红细胞。未观察到不良事件。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Efficacy of lenalidomide in the treatment of myelodysplastic/myeloproliferative neoplasms, unclassifiable (MDS/MPN-U) with the presence of deletion 5q– and trisomy 8+
Myelodysplastic/myeloproliferative neoplasms (MDS/MPN) are very rare clonal myeloid characterized by the simultaneous presence of both of erythrocytic and/orgranulocytic dysplasia with myeloproliferative features. The etiology of the disease remains unknown. The MDS/MPN group is made up of five disorders: chronic myelomonocytic leukemia, atypical chronic myeloid leukemia, juvenile myelomonocytic leukemia, MDS/MPN ring sideroblasts with thrombocytosis and MDS/MPN, un classifiable (MDS/MPN-U). MDS/MPN-U remains the leastcharacterized entity. It accounts less than 5% of all myeloid disorder. The reare currently no well-established diagnostic criteria. The disease is heterogeneous with outclear management strategies. The aim of thispaperis to present a case of a 59-year-old female patient diagnosed with MDS/MPN-U with deletion 5q– and trisomy 8+, whom lenalidomide 10 mg daily has been used as monotherapy. The disease has initially manifested as severe cytopenias, multiple transfusions of blood preparations were required. Currently patientis in the 9th month of treatment. Hemoglobin, platelets and white blood cells returned to normal. The patient completely became independent of transfusion of red blood cell concentrates. No adverse events were observed.
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