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本文报告5例不同类型的原发性免疫缺陷病,临床上均以反复或重症感染为主要表现,生前因均未考虑免疫缺陷的诊断,虽经多种抗菌素治疗,但终因重症机会性感染而致死,其中例3合并恶性网状细胞增生症。尸检发现本组病例的中枢及周围淋巴组织呈不同程度的发育不良,根据其免疫器官的病变,例1～3属重症联合免疫缺陷病范踌,例4、5属B细胞缺陷病。结合本组病例的临床病理特点,对原发性免疫缺陷病的临床表现、诊断及治疗原则进行了讨论。
This article reports 5 cases of different types of primary immunodeficiency disease, clinical manifestations of recurrent or severe infections are the main manifestations of prenatal care without considering the diagnosis of immunodeficiency, although by a variety of antibiotics, but the end result of severe opportunistic infections And died, of which case 3 merged with malignant reticular cell hyperplasia. Autopsy found that central and peripheral lymphoid tissue in this group showed varying degrees of dysplasia, according to the lesions of their immune organs, 1 to 3 cases of severe combined immunodeficiency disease paradox, 4,5 cases of B cell deficiency. Combined with the clinical and pathological features of this group of patients, the clinical manifestations, diagnosis and treatment of primary immunodeficiency disease were discussed.