IgG4相关疾病合并自身免疫性溶血性贫血1例  

IgG4-related diseases with autoimmune hemolytic anemia:A case report

作　　者：彭曼轩 杨丽珍 吴尚洁[1] PEND Manxuan;YANG Lizhen;WU Shangjie(Department of Pulmonary and Critical Care Medicine,Second Xiangya Hospital,Central South University,Changsha 410011,China)

机构地区：[1]中南大学湘雅二医院呼吸与危重症医学科,长沙410011

出　　处：《中南大学学报（医学版）》2020年第6期739-744,共6页Journal of Central South University ：Medical Science

摘　　要：IgG4相关疾病(IgG4-related disease,IgG4-RD)是一种罕见的自身免疫性纤维化疾病,其特征为血清IgG4升高并伴有IgG4阳性细胞浸润的组织器官,可累及多个器官,导致器官肿大、组织损伤。目前糖皮质激素为IgG4-RD的一线治疗方案。自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)也是一种相对少见的由自身反应性红细胞抗体引起的疾病。IgG4-RD和AIHA虽然都是自身免疫相关性疾病,但很少重叠,且它们之间的关系尚未明确。现报告1例IgG4-RD合并AIHA患者。该患者有活动后气促、咳嗽、咳痰;体格检查见贫血貌,皮肤巩膜黄染,颈部可扪及多个肿大淋巴结;该患者经实验室检查、骨髓活检、淋巴结活检确诊。临床医师应开拓思路,提高对此类疾病的认识。IgG4-related disease(IgG4-RD)is a rare autoimmune fibrosis disease characterized by elevated serum IgG4 and tissues as well as organs infiltrated with IgG4-positive cells,resulting in swelling and damage.It is currently treated as first-line treatment with glucocorticoids.Autoimmune hemolytic anemia(AIHA)is also a relatively rare disease that caused by autoreactive erythrocyte antibodies.Although both are autoimmune-related diseases,they rarely overlap.The relationship between them is not clear.A case of IgG4-RD combined with AIHA is reported.The patient has shortness of breath,cough,and sputum after physical activity.Physical examination showed appearance of anemia,yellow staining of skin and sclera,palpable neck and multiple swollen lymph nodes.Laboratory examination,bone marrow biopsy,and lymph node biopsy confirmed the diagnosis.Therefore,clinicians should develop ideas and raise awareness of such diseases.

关 键 词：IgG4相关疾病 自身免疫性溶血性贫血 IGG4 糖皮质激素 

分 类 号：R556.62[医药卫生—血液循环系统疾病] R593.2[医药卫生—内科学]