【以金黄色葡萄球菌败血症为首发表现的常见变异性免疫缺陷】。

Saira Marlene Cabrera-Arias, A Díaz Terríquez, Z A López Morales, L Berrón Ruiz, B Bayardo Gutiérrez, M Núñez Núñez
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引用次数: 0

摘要

简介:常见变异性免疫缺陷(CVID)是成人中最常见的症状性免疫缺陷,在病因不明的低丙种球蛋白血症病例中通过排除诊断。其表现从复发性感染到自身免疫和恶性肿瘤风险不等。病例报告:一名先前健康的11岁女性患者最初来自并居住在哈利斯科州阿兰达斯的兰乔·埃尔诺加尔,该社区约有250名居民。没有明显的家族史;在家族中没有已知的血缘关系或遗传疾病的存在。结果:左腿外伤后,她患上了骨髓炎。入院时,发现全血细胞减少,淋巴结肿大,肝脾肿大,金黄色葡萄球菌培养阳性脓肿。一种检测先天性免疫错误的方法被启动,揭示免疫球蛋白G和A水平下降。由于全身感染严重,静脉注射免疫球蛋白1 g / kg,扩大免疫抑制研究。检测到淋巴细胞亚群异常,CD19+计数减少:71 mm3, CD3+计数:915 mm3, CD4+ CD45RA+ T细胞(幼稚):92 mm3,总记忆B细胞(3%),非同型转换(2.5%),同型转换(0.5%),浆母细胞(0.3%),CD21计数减少(9%)。该患者被分类为Freiburg 1B共同可变免疫缺陷。开始每月静脉注射免疫球蛋白,剂量为每公斤400毫克。患者对免疫球蛋白治疗反应良好,无后续严重感染。她目前情况稳定,正在接受免疫学家的监测。结论:CVID虽然多见于成人,但也可出现在儿童中。金黄色葡萄球菌脓毒症作为初始表现,如本病例,应引起对潜在免疫缺陷的关注。该病例强调了怀疑严重感染患者原发性免疫缺陷的重要性,强调了早期诊断和治疗以优化预后的必要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Staphylococcus aureus sepsis as an initial manifestation of common variable immunodeficiency].

Introduction: Common variable immunodeficiency (CVID) is the most common symptomatic immunodeficiency in adults, diagnosed by exclusion in cases of hypogammaglobulinemia without an identifiable cause. Its manifestations range from recurrent infections to autoimmunity and risk of malignancy.

Case report: A previously healthy 11-year-old female patient was originally from and resides at Rancho El Nogal in Arandas, Jalisco, a community of approximately 250 inhabitants. There was no significant family history; there was no known consanguinity or presence of genetic diseases in the family. Outcome: After trauma to the left leg, she developed osteomyelitis. Upon admission, pancytopenia, enlarged lymph nodes, hepatosplenomegaly, and abscesses with positive cultures for Staphylococcus aureus were detected. An approach to detecting inborn errors of immunity was initiated, revealing decreased immunoglobulin G and A levels. Due to the severe systemic infection, intravenous immunoglobulin was administered at 1 gram/kilogram, and the immunosuppression study was expanded. Abnormalities in the lymphocyte subpopulation were detected, with decreased CD19+ counts: 71 mm3, CD3+ counts: 915 mm3, CD4+ CD45RA+ T cells (naive): 92 mm3, total memory B cells (3%), non-isotype-switched (2.5%), isotype-switched (0.5%), plasmablasts (0.3%), and decreased CD21 counts (9%). The patient was classified as Freiburg 1B common variable immunodeficiency. Monthly intravenous immunoglobulin was started at a dose of 400 milligrams/kilogram. The patient responded favorably to immunoglobulin treatment, with no subsequent serious infections. She remains stable and is being monitored by immunologists.

Conclusion: CVID, although more common in adults, can present in children. S. aureus sepsis as an initial manifestation, as in this patients case, should raise concerns about possible underlying immunodeficiencies. This case highlights the importance of suspecting primary immunodeficiencies in patients with severe infections, underscoring the need for early diagnosis and treatment to optimize prognosis.

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