特发性多中心型Castleman病伴嗜酸性粒细胞增多1例及文献复习

Idiopathic multicentric Castleman's disease with eosinophilia:a case report and literature review

目的探讨特发性多中心型Castleman病伴嗜酸性粒细胞增多的临床特征、诊治方法及预后。方法总结2015年8月本院收治的1例特发性多中心型Castleman病伴嗜酸性粒细胞增多患者的临床资料及诊治经过,并结合文献复习,探讨该病的发病机制、诊断、治疗及预后。结果患者男,66岁,表现为反复上腹部胀痛、多发淋巴结肿大、贫血、高球蛋白血症、嗜酸性粒细胞增多。PET-CT提示淋巴瘤可能,后经腹股沟淋巴结活检病理检查确诊为Castleman病,经全面分析和检查诊断为特发性多中心型Castleman病伴嗜酸性粒细胞增多。予COP方案化疗1个疗程及CHOP方案化疗2个疗程后,患者临床症状明显缓解,且嗜酸性粒细胞数逐渐下降至正常水平。结论特发性多中心型Castleman病较为罕见,病因尚不清楚,由于缺乏诊断及治疗的指南共识,确诊需结合病理及临床;对于高度怀疑淋巴组织增生性疾病的患者,应尽早行淋巴结活检,避免漏诊、误治。

Objective To explore the clinical feature, diagnosis, therapy and prognosis of idiopathic multicentric Castleman＇s disease（MCD） with eosinophilia. Method 1 case of idiopathic MCD with eosinophilia was studied. The clinical feature, pathogenesis, diagnosis, therapy and prognosis of idiopathic MCD with eosinophilia were further investigated through literature review. Result We present the case of a 66-year-old man manifesting recurrent epigastric pain, multiple lymph node enlargement, anemia, hyper gamma globulinemia and eosinophilia. The PET-CT indicated the possibility of lymphoma. Inguinal lymph node biopsy by pathological examination confirmed the diagnosis of Castleman＇s disease（CD）. The final diagnosis of idiopathic MCD with eosinophilia was made by the whole examination and analysis. The symptoms subsided and the eosinophil count decreased to normal level after chemotherapy with COP and CHOP. Conclusion Idiopathic MCD is extremely rare. Its pathogenesis remained to be unclear.Due to lack of consensus in the diagnosis and treatment, the final diagnosis should be combined with clinical and pathological findings.For the patients suspected to have a lymphoproliferative disorder, lymph node biopsy should be performed as soon as possible, which could avoid misdiagnosis and improper treatment.