[再生障碍性贫血和低风险骨髓增生异常肿瘤的诊断和治疗]。

Takahiro Suzuki
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引用次数: 0

摘要

骨髓增生异常肿瘤(MDS)是由基因突变引起的克隆性血液恶性肿瘤。免疫抑制疗法(IST)对低风险骨髓增生异常性肿瘤(LR-MDS)有效,这些肿瘤的特征包括骨髓增生异常、低囊泡或低环形红细胞、PNH 克隆少量增加或巨核细胞减少。这些 LR-MDS 病例与 AA 的鉴别诊断可能比较困难,精确诊断需要对骨髓细胞性和发育不良进行仔细评估。要决定适当的 LR-MDS 治疗策略,重要的是要评估潜在的病理,并优先选择 IST 作为具有免疫介导的骨髓衰竭特征的患者的一线治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Diagnosis and treatment of aplastic anemia and lower-risk myelodysplastic neoplasms].

Myelodysplastic neoplasms (MDS) are clonal hematological malignancies arising from gene mutations. Immunosuppressive therapies (IST) are effective in lower-risk MDS (LR-MDS) with characteristics such as hypoplastic marrow with low blasts or low ring sideroblasts, and with a small increase of PNH clones or decrease of megakaryocytes. Differential diagnosis of these LR-MDS cases from AA can be difficult, and precise diagnosis requires careful evaluation of bone marrow cellularity and dysplasia. To decide on an appropriate treatment strategy for LR-MDS, it is important to evaluate the underlying pathology, and preferentially select IST as first-line therapy in patients with features that indicate immune-mediated bone marrow failure.

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