11例造血干细胞移植相关血栓性微血管病患者临床特征分析

Clinical characteristics for 11 cases of TA-TMA after HSCT

目的分析造血干细胞移植(HSCT)后移植相关血栓性微血管病(TA-TMA)患者的临床特征。方法选取行HSCT的患者185例,确诊11例TA-TMA患者,作为病例组。从185例患者中随机选取30例年龄、移植方式、人类白细胞抗原配型相合的非TA-TMA患者,作为对照组。比较2组破碎红细胞比例,并分析11例TA-TMA患者的实验室指标结果及临床特征。结果在185例行HSCT的患者中,确诊11例(5.9%,11/185)TA-TMA患者,年龄33(7～45)岁,移植后发病时间76(22～152)d。病例组破碎红细胞比例为3.3%,高于对照组(0.4%)(P<0.05)。11例TA-TMA患者血红蛋白(Hb)水平、血小板(PLT)计数呈进行性降低、减少。乳酸脱氢酶(LDH)、血清肌酐(Cr)、血清总胆红素(TB)、C反应蛋白(CRP)、降钙素原(PCT)、环孢素A(CsA)水平均升高。8例伴有急、慢性移植物抗宿主病(GVHD),6例患者24 h尿蛋白阳性。除1例患者未检出病毒DNA之外,其他患者均伴有1种及以上病毒血症,至随访结束仅4例存活。结论TA-TMA是骨髓移植后极为严重的一种并发症,患者如果合并GVHD,则预后较差,综合多项实验室指标结果有利于TA-TMA的早期诊断、治疗,降低死亡率。

Objective To analyze the clinical characteristics of transplantation-associated thrombotic microangiopathy(TA-TMA)after hematopoietic stem cell transplantation(HSCT).Methods A total of 185 patients undergoing HSCT were enrolled.Totally,11 cases were confirmed as TA-TMA(case group),and 30 non-TA-TMA cases with similar age,transplantation ways and human leukocyte antigen matching donor were enrolled as control group.The ratios of broken red blood cells in peripheral blood between the 2 groups were compared,and the laboratory indicators and clinical characteristics of 11 cases of TA-TMA were analyzed.Results Of the 185 patients undergoing HSCT,11 cases(5.9%,11/185)were TA-TMA.The age was 33(7-45)years old,and the onset time after transplantation was 76(22-152)d.The ratio of broken red blood cells in case group was 3.3%,which was higher than that in control group(0.4%)(P<0.05).In the 11 cases of TA-TMA,hemoglobin(Hb)level and platelet(PLT)count decreased progressively,and lactate dehydrogenase(LDH),serum creatinine(Cr),serum total bilirubin(TB),C-reactive protein(CRP),procalcitonin(PCT)and cyclosporin A(CsA)were at high levels.There were 8 cases with acute and chronic graft-versus-host diseases(GVHD)and 6 cases with positive 24 h urinary protein.Except 1 case was not detected for virus DNA,and the other cases were associated with more than 1 type of virus disease.Only 4 cases were alive until the end of follow-up period.Conclusions TATMA is an extremely serious complication after bone marrow transplantation.The prognosis is poor,when GVHD is combined.The determinations of a number of early laboratory indicators are necessary for the early diagnosis and treatment of TA-TMA to decrease mortality.