ANCA相关性血管炎合并血小板减少的临床特点分析

Clinical Characteristics in ANCA-Associated Vasculitis with Thrombocytopenia

目的对合并血小板减少的抗中性粒细胞胞浆抗体(ANCA)相关性血管炎(AAV)患者的临床特点进行分析,以提高对该临床合并症的认识。方法现收集郑州大学第一附属医院2010年1月至2019年9月期间住院的17例AAV合并血小板减少患者的临床资料(血小板减少组),并与同期未合并血小板减少的51例患者(血小板正常组)对比分析其临床特点。结果血小板减少组骨髓穿刺结果提示:1例患者骨髓抑制,1例患者骨髓穿刺结果未报告骨髓巨核细胞数值,骨髓巨核细胞数目为84(0~200)个,且4例血小板抗体阳性。血小板减少组出现咯血、胸腔积液、腹腔积液比例均高于血小板正常组,血红蛋白、C3、白蛋白水平均低于血小板正常组,CRP水平、c-ANCA阳性率高于血小板正常组,MPO-ANCA水平、p-ANCA阳性率均低于血小板正常组(均P<0.05)。17例AAV合并血小板减少的患者均采用糖皮质激素治疗,6例联合环磷酰胺治疗,6例联合血浆置换治疗,2例联合丙种球蛋白治疗。治疗后8例好转,7例死亡,2例因预后差,自动出院后失访。其中8例合并肾脏受累的患者,3例单用糖皮质激素治疗后2例死亡,5例患者联合血浆置换治疗后均好转。结论免疫性血小板减少可能继发于AAV患者,血小板减少可能是AAV预后不良的标志,当患者出现血小板减少的时候更容易出现咯血、腹腔积液、胸腔积液、CRP升高、C3下降和低白蛋白血症。

Objective To analyze the clinical characters in ANCA associated vasculitis(AAV)patients with thrombocytopenia in order to improve the understanding of the clinical complications.Methods The clinical data of 17 patients with AAV complicated with thrombocytopenia(thrombocytopenia group)who were hospitalized in the First Affiliated Hospital of Zhengzhou University from January 2010 to September 2019 were collected and compared with 51 patients without thrombocytopenia in the same period(platelet normal group)to analyze their clinical characteristics.Results The results of bone marrow puncture in thrombocytopenia group showed that:1 patient had myelosuppression,1 patient had no megakaryocytes in bone marrow,the number of megakaryocytes in bone marrow was 84(0-200),and 4 patients were positive for platelet antibody.Compared with the platelet normal group,the proportion of hemoptysis,pleural effusion,peritoneal effusion were significantly increased,hemoglobin level,C3 level,albumin were significantly decreased,CRP,c-ANCA positive rate were significantly increased,MPO-ANCA level,p-ANCA positive rate were significantly decreased(all P<0.05).Seventeen patients with AAV complicated with thrombocytopenia were treated with glucocorticoids,6 cases with cyclophosphamide,6 cases with plasma exchange,2 cases with intravenous immunoglobulin.After treatment,8 cases recovered,7 cases died and 2 cases were discharged from hospital automatically because of poor prognosis,and then lost contact.Among them,8 patients with renal involvement,2 patients died after treatment with glucocorticoid alone,and 5 patients improved after treatment with plasma exchange.Conclusion Immune thrombocytopenia may be secondary to AAV.Thrombocytopenia may be a sign of poor prognosis of AAV.It is more likely to occur hemoptysis,peritoneal effusion,pleural effusion,increased CRP,decreased C3 and hypoalbuminemia,When AAV is combined with thrombocytopenia.