目的 分析血栓性血小板减少性紫癜(thrombotic thrombocytopenic purpura,TTP)的临床特征、诊断、治疗及转归.方法 回顾性分析2013年5月至2022年8月福建省立医院收治的20例TTP患者的一般资料、临床表现、实验室指标、治疗方案及转归.结果 20例TTP患者中男性12例(60％),女性8例(40％),平均年龄(50.95±18.54)岁.具有"五联征"者11例(55％),"三联征"者19例(95％).15例检测了血浆血管性血友病因子裂解酶13(a disintegrin-like and metalloproteinase with thrombospondin type 1 motif,member 13,ADAMTS13)活性的TTP患者中,12例酶活性<10％,其中PLASMIC评分6～7分者11例(91.7％).20例TTP患者中,17例给予甲泼尼龙治疗,其中14例联合了血浆置换、4例联合间断血浆输注、7例联合丙种球蛋白、4例联合环磷酰胺、4例联合利妥昔单抗治疗;1例给予血浆置换联合间断血浆输注治疗;1例仅给予甲泼尼龙治疗;2例放弃治疗.最终死亡7例(35％),存活13例(65％).结论 TTP临床表现复杂多样,以"三联征"多见;ADAMTS13酶活性检测及PLASMIC评分有助于TTP的早期诊断;宜及时进行血浆置换并联合糖皮质激素治疗以期降低TTP患者死亡率.
Clinical analysis of 20 patients with thrombotic thrombocytopenic purpura
Objective To analyze the clinical features,diagnosis,treatment and outcome of thrombotic thrombocytopenic purpura (TTP). Method The clinical data and manifestations,laboratory indicators,treatment plan and outcome of 20 patients with TTP admitted to Fujian Provincial Hospital from 2013 to 2022 were retrospectively analyzed. Result Among the 20 patients with TTP,12 (60％) were males and 8 (40％) were females. The average age was (50.95±18.54) years old. There were 11 cases (55％) had pentads and 19 cases (95％) had triads. Of the 15 TTP patients who detected plasma von Willebrand factor lyase activity,12 patients had enzyme activity less than 10％. Eleven of the 12 patients had a PLASMIC score of six to seven. Of the 20 TTP patients,17 were treated with methylprednisolone,of which 14 were treated with plasma exchange,4 with interrupted plasma infusion,7 with gamma globulin,4 with cyclophosphamide,and 4 with rituximab. One case was treated with plasma exchange combined with intermittent plasma infusion. One case was treated with methylprednisolone only. Two cases gave up treatment. Seven (35％) died and thirteen (65％) survived. Conclusion The clinical manifestations of TTP are complex and multifaceted,with the triads being frequently recognized. ADAMTS13 enzyme activity detection and PLASMIC score were helpful for the early diagnosis of TTP. Plasma exchange combined with glucocorticoid therapy is expected to reduce mortality in TTP patients.
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