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骨髓增生异常综合征的骨髓细胞遗传学和细胞培养分析:对病理生理学和预后的见解

Marrow cytogenetic and cell-culture analyses of the myelodysplastic syndromes: insights to pathophysiology and prognosis.

作者信息

Gold E J, Conjalka M, Pelus L M, Jhanwar S C, Broxmeyer H, Middleton A B, Clarkson B D, Moore M A

出版信息

J Clin Oncol. 1983 Oct;1(10):627-34. doi: 10.1200/JCO.1983.1.10.627.

Abstract

Marrow cytogenetic and granulocyte-macrophage colony formation (CFU-GM) studies were performed on 34 previously untreated patients with documented myelodysplastic syndromes seen between January 1978 and June 1982. All patients were managed without chemotherapy until progression to acute leukemia was observed. All 10 patients with exclusively abnormal marrow metaphases developed acute leukemia (100%) while only one (7%) of 14 patients with solely normal marrow metaphases subsequently developed leukemia (p less than 0.001). Three (42%) of the seven patients with both normal and abnormal marrow metaphases developed acute leukemia. Fifteen (86%) of the 19 patients with either large cluster or no growth patterns developed acute leukemia while only two (13%) of 15 patients with either small cluster or colony forming growth patterns developed acute leukemia (p less than 0.001). Abnormal marrow cytogenetic status correlated with abnormal marrow CFU-GM growth pattern (p less than 0.05). Analysis of CFU-GM sensitivity to inhibition by prostaglandin E was performed in 12 patients. Nine patients showed CFU-GM refractoriness to inhibition by prostaglandin E. Seven of these patients eventually developed leukemia. Three patients had CFU-GMs which were initially sensitive to prostaglandin E inhibition. In these three patients, a loss of CFU-GM sensitivity to prostaglandin E was observed prior to their progression to morphologically identifiable acute leukemia.

摘要

对1978年1月至1982年6月期间收治的34例未经治疗且确诊为骨髓增生异常综合征的患者进行了骨髓细胞遗传学和粒细胞-巨噬细胞集落形成(CFU-GM)研究。在观察到疾病进展为急性白血病之前,所有患者均未接受化疗。10例骨髓中期相完全异常的患者均发展为急性白血病(100%),而14例骨髓中期相完全正常的患者中只有1例(7%)随后发展为白血病(p<0.001)。7例骨髓中期相既有正常又有异常的患者中有3例(42%)发展为急性白血病。19例具有大集落或无生长模式的患者中有15例(86%)发展为急性白血病,而15例具有小集落或集落形成生长模式的患者中只有2例(13%)发展为急性白血病(p<0.001)。骨髓细胞遗传学异常状态与骨髓CFU-GM生长模式异常相关(p<0.05)。对12例患者进行了CFU-GM对前列腺素E抑制作用敏感性的分析。9例患者的CFU-GM对前列腺素E的抑制作用表现为耐药。其中7例患者最终发展为白血病。3例患者的CFU-GM最初对前列腺素E抑制敏感。在这3例患者中,在其进展为形态学可识别的急性白血病之前,观察到CFU-GM对前列腺素E的敏感性丧失。

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