Pamphilon D H, Aparicio S R, Roberts B E, Menys V C, Tate G, Davies J A
Scand J Haematol. 1984 Nov;33(5):486-91. doi: 10.1111/j.1600-0609.1984.tb00730.x.
The myelodysplastic syndromes (MDS) are characterised by dysplastic marrow and cytopenia. Clinically detectable bleeding is uncommon and usually attributed to thrombocytopenia. We have investigated some aspects of haemostatic function in 17 patients with MDS and compared the results with findings from 17 control patients matched for age and sex. No specific disorder of blood coagulation or fibrinolysis was identified. The main abnormalities observed in the patients were: prolongation of the bleeding time which was greater than could be explained on the basis of thrombocytopenia in 13 patients; absent, or severely impaired platelet aggregation in response to collagen in 7 patients; impaired platelet production of malondialydehyde when stimulated with collagen and abnormal release of 14C-5 hydroxytryptamine in 5 patients; and abnormalities of ultrastructure in all 5 patients whose platelets were viewed by electron microscopy.
骨髓增生异常综合征（MDS）的特征是骨髓发育异常和血细胞减少。临床上可检测到的出血并不常见，通常归因于血小板减少。我们研究了17例MDS患者止血功能的某些方面，并将结果与17例年龄和性别相匹配的对照患者的研究结果进行了比较。未发现血液凝固或纤维蛋白溶解的特定紊乱。在患者中观察到的主要异常情况有：13例患者的出血时间延长，延长程度超过了血小板减少所能解释的范围；7例患者对胶原的血小板聚集缺失或严重受损；5例患者在胶原刺激下血小板丙二醛生成受损以及14C - 5羟色胺异常释放；在通过电子显微镜观察血小板的所有5例患者中均存在超微结构异常。
