Clinical Manifestations, Lymphocyte Subsets, and Complications of Patients with Common Variable Immunodeficiency in Taiwan

台灣常見性變異性免疫不全的病人的臨床表現，淋巴細胞數目，及併發症的研究

鄭聖翰(Sheng-Han Cheng) ； 藍忠亮(Joung-Liang Lan) ； 謝祖怡(Tsu-Yi Hsieh) ； 陳得源(Der-Yuan Chen)

《Formosan Journal of Rheumatology》 21卷2期 (2007/12) Pp. 40-45

https://doi.org/10.6313/FJR.2007.21(02).07

摘要

目的：分析常見性變異性免疫不全的病人的臨床表現，淋巴細胞數目及其併發症。方法：在這回溯性的研究裡面，包含了12個從1993年到2007年的常見性變異性免疫不全的病人。記錄了臨床的表現及併發症。用流式細胞技術來測量周邊淋巴細胞的種類。結果：12個病人中，8個是男性，4個是女性。其發生常見性變異性免疫不全疾病的平均年齡是38.7歲。免疫球蛋白G,A,M的平均數值是277.8，25.6及34。在8個有測量淋巴細胞數目的病人中，其中7個的B細胞數目是不足的，其中的6個病人的CD8T細胞是上升的。12個病人的反覆性的感染，包括有肺炎，鼻竇炎，泌尿道感染，菌血症。6個病人有慢性肺疾病，像是支氣管擴張症。三個病人有自體免疫的現象，4個病人有腫瘤疾病。結論：我們研究的常見性變異性免疫不全的病人有比較高風險得到反覆性的感染，慢性肺疾病，腫瘤疾病，自體免疫的現象，其中自體免疫的現象包括有修格連氏症候群及抗磷脂抗症候群。

關鍵字

常見性變異性免疫不全 ； 臨床表現 ； 併發症 ； 淋巴細胞數目

並列摘要

Objective: To analyze the clinical features, lymphocyte subsets, and complications of patients with common variable immunodeficiency (CVID).
Methods: In this retrospective study, twelve patients with CVID diagnosed between 1993 and 2007 were enrolled. Clinical features as well as complications were recorded. Lymphocyte subsets in the peripheral blood (PB) were analyzed using flow cytometry.
Results: Eight patients (66.7%) were male and four patients (33.3%) were female and the mean age at onset was 38.7±9.4 years. The mean levels of serum immunoglobulin (Ig) G, IgA and IgM were 277.8±201.3 mg/dL, 25.6±17.4 mg/dL, and 34.0±17.2 mg/dL, respectively. Markedly decreased percentage of B cells was noted in 7 (87.5%) patients with CVID and elevated percentage of CD8+T cell was found in 6 (75%) patients after analysis of lymphocyte subsets. Eleven of 12 had recurrent infection, including pneumonia, sinusitis, urinary tract infection and septicemia. Six of 12 had chronic lung disease, such as bronchiectasis. Three patients had autoimmune phenomenon and 4 had tumor.
Conclusion: Our CVID patients had a higher risk of recurrent infection, chronic lung disease, tumor, and autoimmune phenomenon, including Sjogren's syndrome and antiphospholipid antibody syndrome (APS).

並列關鍵字

Common variable immunodeficiency ； clinical manifestation ； complications ； lymphocyte subsets