选择性IgA缺乏伴多种自身免疫性合并症:1例报告及文献回顾

IF 1.1 4区 医学 Q4 IMMUNOLOGY Iranian Journal of Immunology Pub Date : 2023-05-31 DOI:10.22034/iji.2023.97452.2513
Liang Hua, Dongjie Guo, Xin Liu, Jiaqi Jiang, Qian Wang, Yi Wang, Te Liu, Fulun Li
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引用次数: 0

摘要

病例:选择性免疫球蛋白- a缺乏症(SIgAD)患者通常无症状,有症状的SIgAD患者通常有自身免疫合并症。一个48岁的汉族男性表现为腹部不适,便血,并在肛门生殖器区域的一个大肿瘤。根据患者年龄、血清IgA浓度(0.067 g/L)和慢性呼吸道感染的证据,初步诊断为SIgAD。没有其他免疫球蛋白缺乏或免疫抑制的证据。对巨大尖锐湿疣的初步诊断是基于人类乳头瘤病毒6阳性的实验室结果和组织学特征。切除肿瘤及邻近皮损。血红蛋白浓度降至5.50 g/dL,并进行了紧急红细胞输血。体温升高至39.8℃,提示输液反应,静脉给予地塞米松5 mg。血红蛋白浓度稳定在10.5 g/dL。临床体征和实验室结果提示自身免疫性溶血性贫血、系统性红斑狼疮和桥本甲状腺炎。腹部不适及便血消退。虽然不常见,但SIgAD患者可出现多种自身免疫合并症的表现。SIgAD的病因和常作为合并症出现的自身免疫性疾病需要进一步的研究。
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Selective IgA Deficiency with Multiple Autoimmune Comorbidities: A Case Report and Literature Review.

Case: Individuals with Selective Immunoglobulin-A Deficiency (SIgAD) are often asymptomatic, and symptomatic SIgAD patients often have autoimmune comorbidities. A 48-year-old Han Chinese man presented with abdominal discomfort, hematochezia, and a large tumor in the anogenital region. The primary diagnosis of SIgAD was based on the patient's age, serum IgA concentration (0.067 g/L), and the evidence of chronic respiratory infection. No other immunoglobulin deficiency or evidence of immunosuppression was present. The primary diagnosis of giant condyloma acuminatum was based on human papilloma virus-6-positive laboratory results and histological characteristics. The tumor and adjacent skin lesions were resected. Hemoglobin concentration fell to 5.50 g/dL, and an emergency erythrocyte transfusion was performed. The body temperature increased to 39.8 ºC, suggesting a transfusion reaction, and 5 mg dexamethasone was administered intravenously. Hemoglobin concentration stabilized at 10.5 g/dL. The clinical signs and laboratory results indicated autoimmune hemolytic anemia, systemic lupus erythematosus, and Hashimoto's thyroiditis. Abdominal discomfort and hematochezia subsided. Though uncommon, the manifestation of multiple autoimmune comorbidities can occur in SIgAD patients. Further research is needed regarding the causes of SIgAD and the autoimmune disorders that often occur as comorbidities.

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来源期刊
Iranian Journal of Immunology
Iranian Journal of Immunology Medicine-Immunology and Allergy
CiteScore
1.60
自引率
0.00%
发文量
50
审稿时长
12 weeks
期刊介绍: The Iranian Journal of Immunology (I.J.I) is an internationally disseminated peer-reviewed publication and publishes a broad range of experimental and theoretical studies concerned with all aspects of immunology.
期刊最新文献
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