嘌呤核苷酸分解过程中遗传性酶缺损与胸腺依赖性(T)和胸腺不依赖性(B)淋巴细胞功能的免疫缺损疾病有关。作者在一组表现低γ-球蛋白血症的不同患者中,观察到淋巴细胞外表面上5′-核苷酸酶活性降低。为了区别5′-核苷酸酶缺损是否与淋巴细胞功能异常的某一特殊形式有关,作者测定了8例患先天性无γ-球蛋白血症的男性患者的外-5′-核苷酸酶,证明全部8例患者淋巴细胞外- Genetic enzyme defects during purine nucleotideolytic processes are associated with immunodeficiency diseases that are thymus-dependent (T) and thymus-independent (B) lymphocyte functions. In a series of different patients exhibiting low [gamma] -globulinemia, a decrease in 5’-nucleotidase activity on the outer surface of lymphocytes was observed. To distinguish whether 5’-nucleotidase deficiency is associated with a particular form of lymphocyte dysfunction, the authors measured the 5’-nucleotides in 8 male patients with congenital absence of [gamma] -globulinemia Enzymes, proving that all eight patients had extra-lymphocyte-