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目的分析1例低蛋白血症伴NK细胞减少患儿的临床和免疫学特征。方法详细分析患儿临床资料,并结合实验室血常规检查、淋巴细胞亚群分析、中性粒细胞功能、细胞因子以及淋巴细胞增殖功能分析,来探讨其免疫学特征。结果收住院的1例3岁男性患儿的主要临床表现是反复发作的发热、腹痛和部分性肠梗阻。免疫学检查显示低丙种球蛋白血症、B细胞和NK细胞绝对计数降低和B细胞功能受损。患儿曾出现肠系膜淋巴结肿大和盲肠肿块,影像学显示回盲部结核感染。患儿病程中曾出现巨细胞病毒和真菌感染,这两种感染对于单纯抗体缺陷的原发性免疫缺陷病并不常见。对该患儿随访4年,期间患儿出现反复发作的牙周炎并导致牙齿脱落。结论目前该患儿尚无法明确是何种类型的原发性免疫缺陷病,根据其特异性的临床表现和免疫学特征,可能为某种以抗体缺陷为主的原发性免疫缺陷病。
Objective To analyze the clinical and immunological characteristics of 1 child with hypoproteinemia and NK cell depletion. Methods The clinical data of children were analyzed in detail, and their immunological characteristics were discussed in combination with laboratory blood tests, lymphocyte subsets analysis, neutrophil function, cytokines and lymphocyte proliferation. Results The main clinical manifestations of 1-year-old boy admitted to hospital were recurrent fever, abdominal pain and partial ileus. Immunological examinations showed hypogammaglobulinemia, decreased absolute B and NK cell counts, and impaired B cell function. Children have had mesenteric lymph nodes and cecum mass, imaging showed ileocecal tuberculosis infection. Cytomegalovirus and fungal infections have occurred in the course of the disease in children, both of which are uncommon for primary immunodeficiency disorders that are deficient in antibody alone. The children were followed up for 4 years, period of recurrent periodontitis occurred in children and lead to tooth loss. Conclusion At present, it is not clear what kind of primary immunodeficiency disease is present in this child. According to its specific clinical manifestations and immunological characteristics, it may be a kind of primary immunodeficiency disease characterized by antibody deficiency.