Sorensen R U, Halpin T C, Abramowsky C R, Hornick D L, Miller K M, Naylor P, Incefy G S
Clin Exp Immunol. 1985 Jan;59(1):217-26.
We have evaluated the immunological abnormalities present in a 6 year old patient with primary intestinal and generalized lymphangiectasia confirmed by intestinal, lung and lymph node biopsies. Lymphocyte loss through the gut was confirmed by the detection of lymphocytes in her stool. An increased enteric protein loss was suggested by hypoproteinaemia, peripheral oedema, and a very short half-life for i.v. immune serum globulin (3 days). Lymphocyte subpopulation analysis revealed a selective loss of T lymphocytes, with a proportionally increased loss of the OKT4 positive helper/inducer subpopulation. Functionally, there was a decrease in proliferative responses to some mitogens and to allogeneic cells, and a lack of T cell help for in vitro B lymphocyte differentiation into immunoglobulin secreting cells. Natural killer function was normal. In this patient, a concomitant thymic deficiency was documented by failure to identify thymic tissue on a thymus biopsy and by an absence or decrease of the serum thymic factor (thymulin) and thymosin alpha 1. No compensatory lymphopoiesis was detected in the bone marrow. In an attempt to increase T lymphocyte development, the patient was treated with thymosin fraction 5. Daily treatment with this preparation resulted in a transient clinical improvement which could not be sustained on a weekly thymosin treatment schedule. However, lymphocyte numbers did not increase during this treatment. The findings in this patient support the notion that T lymphocytes are needed to stimulate thymic epithelium. In situations of excessive loss of long lived T lymphocytes a secondary thymic atrophy may occur and further contribute to the development of a deficiency in cell-mediated immunity.
我们评估了一名6岁原发性肠道和全身性淋巴管扩张症患者存在的免疫异常情况，该诊断经肠道、肺部和淋巴结活检得以证实。通过检测其粪便中的淋巴细胞，证实了淋巴细胞经肠道流失。低蛋白血症、外周水肿以及静脉注射免疫血清球蛋白的半衰期极短（3天）提示肠道蛋白丢失增加。淋巴细胞亚群分析显示T淋巴细胞选择性丢失，其中OKT4阳性辅助/诱导亚群的丢失比例相应增加。在功能上，对某些有丝分裂原和同种异体细胞的增殖反应降低，并且缺乏T细胞对体外B淋巴细胞分化为免疫球蛋白分泌细胞的辅助作用。自然杀伤功能正常。在该患者中，胸腺活检未能发现胸腺组织，血清胸腺因子（胸腺素）和胸腺素α1缺乏或减少，证明存在胸腺缺陷。骨髓中未检测到代偿性淋巴细胞生成。为了增加T淋巴细胞的发育，该患者接受了胸腺素5组分治疗。每日使用该制剂治疗导致临床症状短暂改善，但按每周一次的胸腺素治疗方案则无法维持。然而，在此治疗期间淋巴细胞数量并未增加。该患者的研究结果支持这样一种观点，即需要T淋巴细胞来刺激胸腺上皮。在长寿T淋巴细胞过度丢失的情况下，可能会发生继发性胸腺萎缩，并进一步导致细胞介导免疫缺陷的发展。
