Secretory component deficiency. A disorder of the IgA immune system.
Secretory component deficiency. A disorder of the IgA immune system.
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分泌成分缺乏。
DOI：
10.1056/nejm197602122940701
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发表时间：
1976
期刊：
影响因子：
--
通讯作者：
David L. Nelson
中科院分区：
文献类型：
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作者：
Warren Strober;R. Krakauer;H. Klaeveman;Herbert Y. Reynolds;David L. Nelson
我们研究了一名患有慢性肠道念珠菌病的 15 岁男孩，他的血清 IgA 水平正常，但分泌物中没有 IgA。携带表面 IgA 的外周血淋巴细胞数量增加。此外，用美洲商陆有丝分裂原体外培养的淋巴细胞产生IgA以及其他免疫球蛋白。尽管有证据表明 IgA 合成能力正常，但患者唾液和空肠液中的 IgA 水平大大降低，并且根据 14C-L-亮氨酸掺入估计，患者无法在肠粘膜部位局部合成 IgA。最后，与正常人和 IgA 缺乏症患者相比，患者的唾液或空肠液中没有检测到游离分泌成分。这种疾病的基础可能是 IgA 前体细胞归巢至分泌位点或 IgA 细胞在这些位点选择性增殖/分化的缺陷。
We studied a 15-year-old boy with chronic intestinal candidiasis who had normal serum IgA levels without IgA in his secretions. There was an elevated number of peripheral blood lymphocytes bearing surface IgA. In addition, the lymphocytes cultured in vitro with pokeweed mitogen produced IgA as well as other immunoglubulins. Despite this evidence of normal IgA synthetic capacity, the patient had greatly diminished levels of IgA in the saliva and jejunal fluid, and, as estimated by 14C-L-leucine incorporation, could not synthesize IgA locally at intestinal-mucosal sites. Finally, the patient had no detectable free secretory component in saliva or jejunal fluid in contrast to normal persons and to patients with IgA deficiency. The basis of this disorder is probably a defect in the homing of IgA precursor cells to secretory sites or in the selective proliferation/differentiation of IgA cells at such sites.