血栓性血小板减少性紫癜6例诊治分析

Clinical analysis of diagnosis and treatment of thrombotic thrombocytopenic purpura

目的探讨血栓性血小板减少性紫癜（TTP）早期诊断和治疗方法。方法以6例TTP患者的五联征和血浆疗法为主线进行回顾性临床分析。结果6例TTP患者五联征俱全，以神经、精神征象和血小板减少为突出，外周血中检出红细胞碎片，血清乳酸脱氢酶（LDH）增高。6例患者均接受血浆疗法，其中5例联用糖皮质激素，2例联用长春新碱。5例存活，1例死亡。治疗后4～22d起效，神经、精神征象好转，血清LDH和血小板计数渐趋正常。结论五联征诊断有临床实用性，但需与其他血栓性微血管病、微血管病性溶血性贫血和致血小板减少的疾病作鉴别。早期应用血浆疗法对大多数TTP患者有效，联用免疫抑制剂治疗对提高治愈率和防止复发有重要意义。

Objective To investigate early diagnosis and treatment of thrombotic thrombocypenic purpura （TIP）. Methods Moscheowitz＇s TIP pentad and plasma therapy of 6 TIP patients was retrospectively analyzed. Results The TIP pentad was in all varieties in 6 patients. Neurological signs and thrombocytopenia were protrusive, and the presence of fragment red blood cells （ schistocytes or helmet cells） of microangiopathic hemolysis, serum lactate dehydrogenase （LDH） level was always increased. The plasma therapy was applied in all TIP patients. Corticosteroids were combined in 5 cases. Vincristine was applied in 2 cases. 5 patients survived and 1 patient died. Therapeutic responses occurred after 4-22 days, neurologicalfeatures first improved, in succession serum LDH and blood platelet count gradually recovered. Conclusion In clinical practice the TIP pentad diagnosis has utility value,but to differentiate TIP from TMA, microngiopathic hemolytic anemia（ MHA ） and induced thrombocytopenic disorders is necessary. Early application of plasma therapy is effective for most TIP patients ,and combined immunosuppressive agents has important significance for raising cure rate and preventing relapse.