摘要
目的分析结缔组织疾病合并血栓性血小板减少性紫癜的临床特征、诊断和治疗。方法对5例诊断为结缔组织疾病合并TTP患者一般临床资料、临床特点、实验室检查结果、治疗和转归进行回顾性分析。结果5例均为女性,中位年龄36(21-48)岁,病程中所有患者均有血小板减少和微血管病性溶血性贫血;发热3例;神经系统损害5例,肾功能损害1例;2例患者具有三联征,2例患者具有四联征,1例患者具有五联征。血浆置换(PE)前后血小板(PLT)、血红蛋白(Hb)及乳酸脱氢酶(LDH)监测值有不同,差异有统计学意义(P<0.05),1例复发,利妥昔单抗治疗后缓解,无死亡病例。结论结缔组织疾病与TTP临床表现近似,红细胞形态检查和游离血红蛋白测定对于明确诊断有提示意义,有助于早期识别和诊断,且早期诊断并及时应用糖皮质激素联合血浆置换明显改善患者症状和实验检查结果,难治性或复发性TTP建议使用免疫抑制剂和利妥昔单抗可能改善患者预后。
Objective Analysis of clinical features,diagnosis and treatment of desmosis with thrombotic thrombocytopenic purpura.Methods A retrospective analysis was made on the general clinical data,clinical features,laboratory findings,treatment and prognosis of 5 patients with desmosis complicated with TTP.Results All five patients were female,with a median age of 36(21-48)years.;All patients had thrombocytopenia and microangiopathic hemolytic anemia,fever in 3 ca-ses,nervous system damage in 5 cases,renal function damage in 1 case,triad in 2 cases,quadruple in 2 cases,and quintuple in 1.The monitoring values of platelet(PLT),hemoglobin(Hb)and lactate dehydrogenase(LDH)were different before and after plasma exchange(PE).The difference was statistically significant(<0.05).One case relapsed,and Rituximab was relieved after treatment.No death occurred.Conclusion The clinical manifestations of desmosis are similar to those of TTP.The morphological examination of red blood cells and the determination of free hemoglobin are helpful for early diagnosis.Early diagnosis and timely application of glucocorticoids combined with plasma exchange can obviously improve the symptoms and laboratory results of patients with TTP and refractory or recurrent TTP.It is suggested that immunosuppressive agents and rituximab may improve the prognosis of patients.
作者
叶重阳
张荣荣
梅清
潘爱军
朱春艳
Ye Chongyan;Zhang Rongrong;Mei Qing;Pan Aijun;Zhu Chunyan(Department of Intensive Care Unit,The second people's Hospital of Hefei,Hefei,Anhui,230001,China)
出处
《辽宁医学杂志》
2020年第1期18-21,共4页
Medical Journal of Liaoning
