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1、原發(fā)性血小板增多癥94例臨床分析臨床血液學(xué)雜志2000年第1期第13卷臨床研究作者:楊仁池華寶來(lái)錢(qián)林牛李大鵬單位:楊仁池(中國(guó)醫(yī)學(xué)科學(xué)院中國(guó)協(xié)和醫(yī)科大學(xué)血液學(xué)研究所血液病醫(yī)院(天津,300020)):華寶來(lái)(中國(guó)醫(yī)學(xué)科學(xué)院中國(guó)協(xié)和醫(yī)科大學(xué)血液學(xué)研究所血液病醫(yī)院(天津,300020)):錢(qián)林生(中國(guó)庚學(xué)科學(xué)院中國(guó)協(xié)和醫(yī)科大學(xué)血液學(xué)研究所血液病醫(yī)院(天津,300020));李大鵬(中國(guó)保學(xué)科學(xué)院中國(guó)協(xié)和供科大學(xué)血液學(xué)研究所血液病傑院(天津,300020))關(guān)鍵詞:血小板增多癥,原發(fā)性;診斷;預(yù)后摘耍:目的:探討原發(fā)
2、性血小板增多癥(ET)患者血栓及曲血的發(fā)生率、各種藥物的療效及患者的預(yù)后。方法:對(duì)我院1980年1月?1998年12月間診治的ET患者進(jìn)行回顧性分析。結(jié)果:94例患者中,男42例,女52例,男:女為0.81:1;就診時(shí)中位年齡50歲(9?77歲)。出血者29例(30.9%),血栓者21例(21.3%),同時(shí)有出血和血栓者6例(6.4%)。就診時(shí)中位血小板數(shù)1202.5X109/L((614?3530)X109/L);35例進(jìn)行了骨髓活檢,其中6例局部有網(wǎng)狀纖維增生。33例進(jìn)行了核型分析,Ph染色體均陰性,其屮5
3、例有核型異常。對(duì)其屮67例隨訪>1年,隨訪時(shí)間中位數(shù)6年(1?16年),3例轉(zhuǎn)變?yōu)楣撬枥w維化,其余病例無(wú)明顯變化。馬利蘭治療22/25例有效;甲異靛治療7/9例有效;拜基治療9/9例有效。結(jié)論:原發(fā)性血小板增多癥患者血栓與出血的發(fā)生率相仿,馬利蘭、疑基麻、甲異靛等均有效,預(yù)后較好。ClinicalAnalysisof94CasesofEssentialThrombocythemiaYANGRen-chiHUABaolaiQIANLin-shenLIDa-peng(InstituteofHematologyand
4、BloodDiseasesHospital,CAMSandPUMC,Tianjin300020)Abstract:Objective:Toexploretheincidenceofthrombosisandhemorrhagein,theefficacyofvariousmedicationsforandtheprognosisofpatientswithessentialthrombocythemia.Methods:Essentialthrombocythemiapatientsdiagnosedinour
5、hospitalduringJanuary1980andDecember1998wereretrospectivelyanalyzed.Results:Ninety-fourpatients(42malesand52femaleswithamedianageof50years)werediagnosedinourhospitalinthisperiod.HemoiThageoccurredin30.9%,thrombosisoccurredin21.3%,and6.4%ofpatientshadbothhemo
6、rrhageandthrombosis.Themedianplateletcountatdiagnosiswas1202.5x109/L(rangefrom614to3530x109/L).Bonemarrowbiopsywasperformedin35casesandlocalfibrosiswasrevealedin6cases.Karyotypeanalysiswasperformedin33cases,Phchromosomewasnegativeinallcasesand5caseshadabnorm
7、alkaryotype.Sixty-sevenpatientswerefollowed-upoveroneyearwithamedianfollow-upperiodof6years(rangefrom1to16years).Threepatientsevolvedintomarrowfibrosis.Twenty-twoof25patientswereresponsivetobusulphan,7/9patientswereresponsivetomesoindigoand9/9patientswereres
8、ponsivetohydroxyurea.Conclusion:Theincidenceofhemorrhageandthrombosisinessentialthrombocythemiapatientshasnodifference.Thepatientsareresponsivetobusulphan,mesoindigoandhydroxyurea.Theprognosisis