Han W, Han Y, Chen J, Ma X, Chen F, Wu X J, Qi J Q, Qiu H Y, Sun A N, Wu D P
The First Affiliated Hospital of Soochow University, Jiangsu Institute of Hematology, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Collaborative Innovation Center of Hematology, Soochow University, Suzhou 215006, China.
Zhonghua Xue Ye Xue Za Zhi. 2016 Aug 14;37(8):666-70. doi: 10.3760/cma.j.issn.0253-2727.2016.08.007.
To analyze the clinical characteristics, treatment and prognosis of 16 allogeneic hematopoietic stem cell transplantation (allo- HSCT)- associated thrombotic microangiopathy (TA- TMA) patients.
The clinical data of 16 TA- TMA cases in 852 patients following allo- HSCT from Jan. 2013 to Jun. 2015 in the First Affiliated Hospital of Soochow University were retrospectively analyzed.
Of all the 852 allo-HSCT recipients, 16 patients were diagnosed as TA-TMA and the 1-year cumulative incidence of TA-TMA was (2.3±0.6)%. Among them, there were 9 males and 7 females, the median age was 41-year-old (12-54), and the median times of diagnosis of TA-TMA were 72 (21- 525) days after HSCT. Additionally, the median platelet counts, hemoglobin, percentage of schistocytes and Lactate dehydrogenase (LDH) levels were 20(11-36) ×10(9)/L, 74(56-99) g/L, 3% (2%- 13%) and 762(309-1 049) U/L, respectively. All 16 cases have normal ADAMTS13 level (over 60%), 10 patients had neurologic dysfunction and elevated creatinine were seen in 7. The major treatment of TA-TMA was withdrawn of calcineurin inhibitors, plasma exchange and corticosteroids. Finally, 8 patients achieved response after treatment and the other patients died of poor response. Compared with TA- TMA who achieved remission after therapy, those who got no response after interventions presented acute GVHD and they had higher schistocytes (62.5% cases>5% vs all cases ≤4%), LDH [826 (674-1 310) U/L vs 636 (309- 941) U/L] and serum creatinine levels [127 (70- 215) μmol/L vs 56 (22- 101) μmol/L].
TA-TMA was a severe complication after allo-HSCT, it could progress to multi-organ injury and was associated with poor outcome, the therapeutic efficacy depends on disease severity and coexisted complications.
分析16例异基因造血干细胞移植（allo-HSCT）相关血栓性微血管病（TA-TMA）患者的临床特征、治疗及预后。
回顾性分析2013年1月至2015年6月苏州大学附属第一医院852例接受allo-HSCT患者中16例TA-TMA病例的临床资料。
在852例allo-HSCT受者中，16例被诊断为TA-TMA，TA-TMA的1年累积发病率为（2.3±0.6）%。其中男性9例，女性7例，中位年龄41岁（12 - 54岁），TA-TMA诊断的中位时间为HSCT后72天（21 - 525天）。此外，血小板计数中位数、血红蛋白、裂体细胞百分比和乳酸脱氢酶（LDH）水平分别为20（11 - 36）×10⁹/L、74（56 - 99）g/L、3%（2% - 13%）和762（309 - 1049）U/L。16例患者的ADAMTS13水平均正常（超过60%），10例患者有神经功能障碍，7例肌酐升高。TA-TMA的主要治疗方法是停用钙调神经磷酸酶抑制剂、血浆置换和使用糖皮质激素。最终，8例患者治疗后获得缓解，其他患者因反应不佳死亡。与治疗后缓解的TA-TMA患者相比，干预后无反应的患者出现急性移植物抗宿主病，且裂体细胞更高（62.５%的病例>5%，而所有病例≤4%）、LDH[826（674 - 1310）U/L对636（309 - 941）U/L]和血清肌酐水平[127（70 - 215）μmol/L对56（22 - 101）μmol/L]。
TA-TMA是allo-HSCT后的严重并发症，可进展为多器官损伤且预后不良，治疗效果取决于疾病严重程度和并存的并发症。
