骨髓增生性疾病止血功能的研究

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1、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患者血小板聚集功能、血浆血管性血友病因子相关抗原及血管性血友病瑞斯托霉素辅因子活性，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、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

2、诱导血小板最大聚集率与正常对照组比较均明显降低(P【关键词】骨髓增生性疾病；血小板聚集功能；止血功能；vonWillebrand因子HematostaticFunctioninMyeloproliferativeDiseasesAbstractThisstudywaspurposedtoinvestigatethechangeofearlyhemostaticfunetioninpatientswithmyeloproliferativediseases(MPD)andtoexploreitssignificanceincombinationwithclinicaldata.T

3、heplateletaggregativefunctionwasmeasuredbyusingristomycin,adenosinediphosphate,collagenandadrenineasinductors,theplasmavonWillebrandfactor-associatedantigen(vWF:Ag)levelwasmeasuredbyenzyme-linkedimmunosorbentassay(ELISA),theplasmavonWellebrandfactor-ristomycincofactor(vWF:Rco)activitywasmeas

4、uredinpatientswithobviouslylowristomycininducedplateletaggregation(RIPA).Theresultsshowedthattheplateletaggregativefunctionobviouslydecreasedinpatients,thereweredistinctdifferencesinmaximalplateletaggregativeratebetweenpatientsandnormalcontrolsinducedbyinductorsrespectively(P).PlasmavWF:Rcoa

5、ctivityinallpatientswithMPDchosenwasinthenormalrange,exceptonepatientwithessentialthrombocytosis(ET)whoseplasmavWF:Rcoactivitywasmuchlowerthannormal.NocorrelationwasfoundbetweenplateletcountandplasmavWF:Aglevelinthepatients(r=-).Nocorrelationwasfoundbetweenplateletcountandmaximalplateletaggr

6、egativerateinducedbyinductorsrespectivelyinpatients.ItisconcludedthattheoccurrenceofabnormalplateletaggregativefunctionishighinpatientswithRIPAandvWF:RcoactivitydecreaseinonepatientwithET.However,theshortageofvWFpolymerexistedinhisplasmahaveneedsforfurtherresearch.Nocorrelationwasobservedbet

7、weenhemostsisandclinical,becauseofthehighoccurrenceofplateletdysfunctioninMPDpatients,theclinicalapplicationofanti-plateletdrugsshouldbeconsideratedcarefully.Keywordsmyeloproliferativedisease;plateletaggregativefunction;hematostaticfunction;vonWill