血浆置换治疗血栓性血小板减少性紫癜临床疗效的观察
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摘要
目的观察血浆置换治疗血栓性血小板减少性紫癜的临床疗效。方法回顾分析2013年1月—2018年6月本院14例确诊为血栓性血小板减少性紫癜患者的临床特征及进行血浆置换治疗后的疾病转归情况,分析血浆置换治疗过程中不良反应发生情况。结果 14例血栓性血小板减少性紫癜患者共进行了47次血浆置换,经过血浆置换治疗后10例患者病情好转恢复出院,血浆置换治疗血栓性血小板减少性紫癜患者的总有效率为71.4%。47次血浆置换术中共发生了3次不良反应,其不良反应发生率为6.4%。结论血浆置换是治疗血栓性血小板减少性紫癜的有效方法,患者一旦确诊或高度怀疑为血栓性血小板减少性紫癜时需尽早开始血浆置换治疗。
Objective To investigate the clinical effect of therapeutic plasma exchange for the treatment of patients with thrombotic thrombocytopenic purpura(TTP). Methods The clinical profiles of 14 patients with TTP and their outcome after plasma exchange in our hospital from January 2013 to June 2018 were analyzed retrospectively.Results In 14 TTP patients, 47 plasma exchange procedures were performed.Ten of 14(71.4%) patients demonstrated a good response to plasma exchange therapy and were discharged from hospital with complete remission. Three adverse reactions occurred in 47 procedures, with the incidence of adverse reactions of 6.4%.Conclusion Plasma exchange is effective for treatment of TTP. Once the patient was diagnosed or highly suspected as TTP, plasma exchange should be initiated as soon as possible.
Objective To investigate the clinical effect of therapeutic plasma exchange for the treatment of patients with thrombotic thrombocytopenic purpura(TTP). Methods The clinical profiles of 14 patients with TTP and their outcome after plasma exchange in our hospital from January 2013 to June 2018 were analyzed retrospectively.Results In 14 TTP patients, 47 plasma exchange procedures were performed.Ten of 14(71.4%) patients demonstrated a good response to plasma exchange therapy and were discharged from hospital with complete remission. Three adverse reactions occurred in 47 procedures, with the incidence of adverse reactions of 6.4%.Conclusion Plasma exchange is effective for treatment of TTP. Once the patient was diagnosed or highly suspected as TTP, plasma exchange should be initiated as soon as possible.
引文
[1] 王吉耀,廖二元,黄从新,等.内科学.2版,北京:人民卫生出版社,2013:867-869.
[2] 张军,顾健.血栓性血小板减少性紫癜发病机制及治疗进展.临床血液学杂志,2016,29(1):73-76.
[3] 中华医学会血液学分会血栓与止血学组.血栓性血小板减少性紫癜诊断与治疗中国专家共识(2012年版).中华血液学杂志,2012,33(11):983-984.
[4] Mariotte E,Azoulay E,Galicier L,et al .Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura):a cross-sectional analysis of the French national registry for thrombotic microangiopathy.Lancet Haemato,2016,3 (5) :e237-e245.
[5] Veyradier A,Obert B,Houllier A,et al .Specific von Willebrand factor-cleaving protease in thrombotic microangiopathies a study of 111 cases.Blood,2001,98(6):1765-1772.
[6] Bianchi V,Robles R,Alberio L,et al.Von Willebrand factor-cleaving protease(ADAMTS13) in thrombocytopenic disorders:a severely deficient activity is specific for thrombotic thrombocytopenic purpura.Blood,2002,100(2):710-713.
[7] Zheng XL,Kaufman RM,Goodnough LT,et al.Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity,inhibitor level,and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura.Blood,2004,103(11) :4043-4049.
[8] Rogers HJ,Allen C.Thrombotic thrombocytopenic purpura:the role of ADAMTS13.Cleveland Clinic J Med,2016,83(8) :597-603.
[9] Reese JA,Muthurajah DS,Kremer Hovinga JA,et al.Children and adults with thrombotic thrombocytopenic purpura associated with severe,acquired Adamts13 deficiency:comparison of incidence demographic and clinical features.Pediatr Blood Cancer,2013,60(10) :1676-1682.
[10] Joly BS,Coppo P,Veyradier A.Thrombotic thrombocytopenic purpura.Blood,2017,129(21) :2836-2846.
[11] 杨俊龙,肖洁,彭涛,等.血浆置换治疗血栓性血小板减少性紫癜2例报告.中国输血杂志,2017,30(11):1293-1295.
[12] George JN,Al-nouri ZL.Diagnostic and therapeutic challenges in the thrombotic thrombocytopenic purpura and hemolytic uremic syndromes.Hematology Am Soc Hematol Educ Program,2012,2012(2):604-609.
[2] 张军,顾健.血栓性血小板减少性紫癜发病机制及治疗进展.临床血液学杂志,2016,29(1):73-76.
[3] 中华医学会血液学分会血栓与止血学组.血栓性血小板减少性紫癜诊断与治疗中国专家共识(2012年版).中华血液学杂志,2012,33(11):983-984.
[4] Mariotte E,Azoulay E,Galicier L,et al .Epidemiology and pathophysiology of adulthood-onset thrombotic microangiopathy with severe ADAMTS13 deficiency (thrombotic thrombocytopenic purpura):a cross-sectional analysis of the French national registry for thrombotic microangiopathy.Lancet Haemato,2016,3 (5) :e237-e245.
[5] Veyradier A,Obert B,Houllier A,et al .Specific von Willebrand factor-cleaving protease in thrombotic microangiopathies a study of 111 cases.Blood,2001,98(6):1765-1772.
[6] Bianchi V,Robles R,Alberio L,et al.Von Willebrand factor-cleaving protease(ADAMTS13) in thrombocytopenic disorders:a severely deficient activity is specific for thrombotic thrombocytopenic purpura.Blood,2002,100(2):710-713.
[7] Zheng XL,Kaufman RM,Goodnough LT,et al.Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity,inhibitor level,and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura.Blood,2004,103(11) :4043-4049.
[8] Rogers HJ,Allen C.Thrombotic thrombocytopenic purpura:the role of ADAMTS13.Cleveland Clinic J Med,2016,83(8) :597-603.
[9] Reese JA,Muthurajah DS,Kremer Hovinga JA,et al.Children and adults with thrombotic thrombocytopenic purpura associated with severe,acquired Adamts13 deficiency:comparison of incidence demographic and clinical features.Pediatr Blood Cancer,2013,60(10) :1676-1682.
[10] Joly BS,Coppo P,Veyradier A.Thrombotic thrombocytopenic purpura.Blood,2017,129(21) :2836-2846.
[11] 杨俊龙,肖洁,彭涛,等.血浆置换治疗血栓性血小板减少性紫癜2例报告.中国输血杂志,2017,30(11):1293-1295.
[12] George JN,Al-nouri ZL.Diagnostic and therapeutic challenges in the thrombotic thrombocytopenic purpura and hemolytic uremic syndromes.Hematology Am Soc Hematol Educ Program,2012,2012(2):604-609.