Demonstration of an intrathymic defect in a case of severe combined immunodeficiency disease.
Demonstration of an intrathymic defect in a case of severe combined immunodeficiency disease.
复制标题
严重联合免疫缺陷病病例中胸腺内缺陷的证明。
DOI：
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发表时间：
1975
影响因子：
158.5
通讯作者：
E. Gelfand
中科院分区：
文献类型：
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作者：
K. Pyke;H. Dosch;M. Ipp;E. Gelfand
已开发了一种体外系统来阐明原发免疫缺陷疾病中细胞缺陷的性质。在人类胸腺上皮细胞单层培养上，对一名患有严重联合免疫缺陷疾病的儿童的外周血淋巴细胞和骨髓细胞进行孵育，结果出现了与绵羊红细胞形成玫瑰花环的细胞群。同样的细胞制剂允许在体外培养后合成抗原特异性的补体依赖抗体，如空斑检测系统所证明的那样。此外，来自同一儿童的胸腺组织在体外培养时提供了成熟的形态和功能证据。实验结果表明，在这种情况下，淋巴前体细胞存在于骨髓中，但由于胸腺组织成熟缺陷而未能分化为功能成熟。
An in vitro system has been developed to elucidate the nature of the cellular defect in primary immunodeficiency diseases. Incubation, on human thymic epithelial monolayer cultures, of peripheral blood lymphocytes and bone-marrow cells from a child with documented severe combined immunodeficiency disease resulted in the appearance of a population of cells that formed rosettes with sheep erythrocytes. The same cell preparation permitted the synthesis of antigen-specific, complement-dependent antibodies after in vitro education, as demonstrated in a plaque assay system. In addition, thymic tissue from the same child gave morphologic and functional evidence of maturation when cultured in vitro. The experimental results suggest that in this case, lymphoid precursor cells were present in the bone marrow but failed to differentiate to functional maturity due to a defect in maturation of thymic tissue.