骨髓增生性疾病止血功能的研究.docVIP

骨髓增生性疾病止血功能的研究 作者：朱筱,裴敏飞,徐敏,张广森 【摘要】 本研究通过测定骨髓增生性疾病（myeloproliferative disease,MPD）患者血小板聚集功能、血浆血管性血友病因子相关抗原（vWF:Ag）及血管性血友病瑞斯托霉素辅因子（vWF:Rco）活性，观察MPD患者初期止血功能的变化，并与临床资料结合探讨实验室检测结果异常的意义。共观察MPD患者３5例，正常对照２０例。以瑞斯托霉素（ris）、二磷酸腺苷(ADP)、胶原(col)及肾上腺素(adr)作为血小板诱聚剂测定血小板聚集功能；采用酶联免疫吸附双抗体夹心法测定血浆vWF:Ag水平；测定瑞斯托霉素诱聚（RIPA）明显降低的6例患者血浆vWF:Rco活性。结果显示：35例患者血小板聚集功能与正常比较明显降低，4种诱聚剂诱导血小板最大聚集率与正常对照组比较均明显降低(Plt;0.001或P=0.002)。血小板聚集功能异常的患者高达71.4%（25/35）；MPD患者血浆vWF:Ag水平较正常对照无明显差别（105.11±47.54% 与 111.88±55.24%,Pgt;0.50）； RIPA明显降低的6例MPD患者血浆vWF:Rco活性测定示1例原发性血小板增多症（essential thrombocytosis,ET）患者明显低于正常水平，其余患者均在正常范围内；患者血小板计数与血浆vWF:Ag之间无相关性（r=－0.180）。患者血小板计数与4种诱聚剂诱导下血小板最大聚集率之间均无明显相关性。结论：MPD患者血小板聚集功能不良发生率高；1例ET患者有RIPA降低和vWF:Rco降低，但其血浆中是否存在vWF多聚物的缺乏，仍需进一步研究证实；研究观察期间未发现止血功能的异常与临床症状的相关性，但MPD患者中血小板功能缺陷的高发生率提示临床使用血小板抑制剂应持谨慎态度。 【关键词】 骨髓增生性疾病；血小板聚集功能；止血功能；von Willebrand因子 　　Hematostatic Function in Myeloproliferative Diseases 　　AbstractThis study was purposed to investigate the change of early hemostatic funetion in patients with myeloproliferative diseases (MPD) and to explore its significance in combination with clinical data. The platelet aggregative function was measured by using ristomycin,adenosine diphosphate, collagen and adrenine as inductors, the plasma von Willebrand factor-associated antigen (vWF:Ag) level was measured by enzyme-linked immunosorbent assay (ELISA), the plasma von Wellebrand factor-ristomycin cofactor (vWF:Rco) activity was measured in 6 patients with obviously low ristomycin induced platelet aggregation (RIPA). The results showed that the platelet aggregative function obviously decreased in 35 patients, there were distinct differences in maximal platelet aggregative rate between patients and normal controls induced by 4 inductors respectively (Plt;0.001,Plt;0.001,P=0.002,Plt;0.001). There was no obvious difference between patients with MPD and healthy controls in plasma vWF:Ag level (Pgt;0.50). Plasma vWF:Rco activity in all 6 patients w