A Case of Aplastic Anemia-Paroxysmal Nocturnal Hemoglobinuria with Poor Response to Complement Inhibitor Treatment:Multidisciplinary Diagnostic and Therapeutic Analysis
10.12376/j.issn.2097-0501.2025.02.010
- VernacularTitle:一例补体抑制剂治疗欠佳的再生障碍性贫血-阵发性睡眠性血红蛋白尿患者的多学科诊疗分析
- Author:
Juan WU
1
;
Di WU
;
Jin XU
;
Bing HAN
;
Guiren RUAN
;
Yindong LIU
;
Peipei CHEN
;
Miao CHEN
;
Min SHEN
Author Information
1. 中国医学科学院北京协和医院全科医学科,北京 100730
- Publication Type:Journal Article
- Keywords:
paroxysmal nocturnal hemoglobinuria;
aplastic anemia;
odontogenic infection;
fever;
rare diseases
- From:
JOURNAL OF RARE DISEASES
2025;4(2):214-220
- CountryChina
- Language:Chinese
-
Abstract:
A young male patient was diagnosed with aplastic anemia accompanied by paroxysmal nocturnal hemoglobinuria(PNH),and achieved only partial remission after immunosuppressive therapy.Over the past year,his anemia worsened,leading to transfusion dependence,which was considered to be caused by active he-molysis of PNH.Despite switching to eculizumab treatment,his anemia continued to deteriorate.A detailed medical history revealed chronic periodontal infection,with periodontal pain,purulent discharge,and fever in the past month.Multidisciplinary discussion suggested that chronic odontogenic infection activating the comple-ment system was the primary cause of aggravated PNH hemolysis,and the acute infection affected the efficacy of eculizumab.Based on multidisciplinary consultation,a treatment plan was formulated,including intravenous antibiotics combined with periodontal irrigation and tooth extraction to control odontogenic infection,while con-tinuing regular eculizumab infusion.The patient's symptoms improved,hemoglobin levels rose rapidly,and he was no longer transfusion-dependent.This case provides an empirical reference for addressing difficulties en-countered in the treatment of rare diseases with new drugs through multidisciplinary collaborative diagnosis and treatment.
