Cherniawsky Hannah, Razavi Habib Moshref
Division of Haematology, Department of Medicine University of British Columbia Vancouver British Columbia Canada.
Division of Hematopathology and Transfusion Medicine, Fraser Health Authority University of British Columbia Vancouver British Columbia Canada.
Clin Case Rep. 2023 May 29;11(6):e7409. doi: 10.1002/ccr3.7409. eCollection 2023 Jun.
No formal treatment guidelines for MDS/MPN-RS-T exist. With salient features such as anemia and thrombocytosis, management is individualized and aims to address anemia, thrombosis, and in some cases acquired von Willebrand's disease.
Myelodysplastic/myeloproliferative overlap syndrome with ring sideroblasts and thrombocytosis (MDS/MPN-RS-T) is a rare myeloid neoplasm showing myelodysplastic and myeloproliferative features. With extremely raised platelets, possibility of acquired von Willebrand and risk of hemorrhage is increased. With this quandary in mind, a descriptive case and a brief discussion of available treatments ensues.
目前尚无骨髓增生异常/骨髓增殖性肿瘤伴环形铁粒幼细胞和血小板增多症(MDS/MPN-RS-T)的正式治疗指南。鉴于存在贫血和血小板增多等显著特征,治疗需个体化,目标是解决贫血、血栓形成问题,在某些情况下还需处理获得性血管性血友病。
骨髓增生异常/骨髓增殖性重叠综合征伴环形铁粒幼细胞和血小板增多症(MDS/MPN-RS-T)是一种罕见的髓系肿瘤,具有骨髓增生异常和骨髓增殖的特征。血小板极度升高会增加获得性血管性血友病的可能性和出血风险。鉴于此困境,本文将介绍一个描述性病例并简要讨论可用的治疗方法。