不同诱因致获得性血栓性血小板减少性紫癜20例临床研究

Clinical study on 20 cases of acquired thrombotic thrombocytopenic purpura caused by different inducements

目的通过分析获得性血栓性血小板减少性紫癜(TTP)患者的临床资料,提高对TTP的认识。方法回顾性分析20例获得性TTP患者的临床特征、实验室检查、治疗策略及转归。结果 20例获得性TTP患者中,16例有诱发因素,包括:自身免疫异常、系统性红斑狼疮、抗磷脂抗体综合征、病毒感染、细菌感染、药物、淋巴瘤及异基因造血干细胞移植。初诊时仅5例(25%)表现为典型"五联"征,18例患者(90%)伴血小板减少,14例(70%)伴微血管病性溶血性贫血,17例(85%)伴神经精神症状,15例(75%)伴发热,8例(40%)伴肾功能不全。16例(80%)网织红细胞百分比升高,18例(90%)检出破碎红细胞,其中3例同时检出小球形红细胞;20例(100%)患者乳酸脱氢酶均升高。20例患者均接受了以血浆疗法(血浆置换或/和血浆输注)为主的治疗,其中9例患者接受了糖皮质激素治疗,2例患者接受了环磷酰胺治疗,2例接受人免疫球蛋白治疗,4例患者接受小剂量利妥昔单抗治疗(100mg每周1次,连用4次)。11例(55%)患者完全缓解,3例(15%)患者部分缓解,总有效率为70%;4例(15%)患者死亡,治疗有效的14例患者中3例(21%)复发。结论 TTP诊断依赖临床资料的综合分析,早期诊断和以血浆疗法为主要手段的早期治疗可改善患者的预后。

Objective To enhance the recognition of thrombotic thrombocytopenic purpura（TTP）by analyzing the clinical data of acquired TTP.Methods The clinical features,laboratory detection,treatment strategy and outcome of 20 patients with acquired TTP were retrospectively analyzed.Results Among 20 patients with acquired TTP,16 cases had the inducing factors,including abnormal autoimmune,systemic lupus erythematosus（SLE）,antiphospholipid antibody syndrome（APS）,viral infection,bacterial infection,medication,lymphoma and allogenic hematopoietic stem cell transplantation.When the patients were diagnosed firstly,only 5cases（25%）had typical pentalogy of TTP,18cases（90%）were complicated with thrombocytopenia,14cases（70%）were complicated with microangiopathic hemolytic anemia,17cases（85%）with neurologic abnormalities,15cases（75%）fever and 8cases（40%）with renal insufficiency.Sixteen cases（80%）had high percentage of reticulocytes,the schistocytes were detected in the peripheral blood smears of 18 cases,among them microspherocytes were simultaneously detected in 3cases.Twenty cases had high level of lactate dehydrogenase.All the cases received the therapy of plasmatherapy（plasma exchange and/or plasma infusion）as the principal thing,among them 9cases received the glucocorticoid therapy,2cases received the cytoxan therapy,2cases received the human immune globulin therapy and 4cases received the low-dose rituximab therapy（100 mg,once per week,for consecutive 4times）.Eleven cases（55%）reached complete remission（CR）,3（15%）cases got partial remission（PR）and 4（15%）cases died.The total effective rate was 70%.Three cases（21%）out of 14 effective cases relapsed.Conclusion The diagnosis of TTP is based on the comprehensive analysis of clinical data.Early diagnosis and early treatment of plasmatherapy as the main means can improve the prognosis of patients with TTP.