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[骨髓增生异常综合征]

[Myelodysplastic syndromes].

作者信息

Giagounidis A

机构信息

Klinik für Onkologie, Hämatologie und Palliativmedizin, Marienhospital Düsseldorf, Rochusstr. 2, 40479, Düsseldorf, Deutschland.

出版信息

Internist (Berl). 2013 Jun;54(6):699-708. doi: 10.1007/s00108-012-3214-3.

Abstract

The myelodysplastic syndromes (MDS) are clonal disorders of hematopoietic stem cells. The transformation rate to acute myeloid leukemia reaches 30-40 %. In early phases of the disease, the clonal cells have a growth advantage but suffer from premature apoptosis, which explains the paradox of a cellular bone marrow coupled to peripheral blood cytopenias. At later stages, additional genetic aberrations accumulate and lead to proliferation with leukemic transformation. Patients with early MDS benefit from supportive therapy or growth factors. Sometimes, immunological or immunomodulatory treatments can suppress the malignant clone and strengthen normal hematopoiesis for sustained periods. Patients with advanced MDS are usually treated with cytotoxic therapy followed by allogeneic stem cell transplantation or with epigenetic therapy to initiate differentiation and slow down proliferation.

摘要

骨髓增生异常综合征(MDS)是造血干细胞的克隆性疾病。转化为急性髓系白血病的发生率达30%-40%。在疾病早期,克隆细胞具有生长优势,但会过早凋亡,这解释了细胞性骨髓伴外周血细胞减少的矛盾现象。在疾病后期,额外的基因畸变会累积,并导致增殖伴白血病转化。早期MDS患者受益于支持性治疗或生长因子。有时,免疫或免疫调节治疗可抑制恶性克隆并在较长时期内增强正常造血。晚期MDS患者通常先接受细胞毒性治疗,随后进行异基因干细胞移植,或接受表观遗传治疗以启动分化并减缓增殖。

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