Consolini R, Cini P, Cei B, Bottone E
Am J Pediatr Hematol Oncol. 1987 Summer;9(2):146-8. doi: 10.1097/00043426-198722000-00007.
In some patients with histiocytosis-X there is a deficiency of suppressor T cells, which is corrected in vitro by incubation with either crude calf thymic extract or thymostimulin. This finding suggests that a thymus deficiency could be involved in this disease. Low levels of serum thymic factor (FTS) are found in patients with histiocytosis-X. Their plasma contain factors capable of inhibiting biological activity of FTS in vitro. Elucidation of the mechanism by which FTS is inhibited would be helpful in understanding the immunological defect in histiocytosis-X. The presence of evidence of thymic dysfunction and the fact that patients respond to thymic hormone therapy suggest that histiocytosis-X could be due to a primary immunodeficiency syndrome. Thymulin, which stimulates the generation of suppressor T cells, could be of benefit in therapy.
在一些组织细胞增多症-X患者中，存在抑制性T细胞缺乏的情况，通过与粗制小牛胸腺提取物或胸腺刺激素一起孵育，可在体外得到纠正。这一发现提示胸腺功能不足可能与该疾病有关。组织细胞增多症-X患者血清胸腺因子（FTS）水平较低。他们的血浆含有能够在体外抑制FTS生物活性的因子。阐明FTS被抑制的机制将有助于理解组织细胞增多症-X中的免疫缺陷。胸腺功能障碍证据的存在以及患者对胸腺激素治疗有反应这一事实表明，组织细胞增多症-X可能是由原发性免疫缺陷综合征引起的。刺激抑制性T细胞生成的胸腺素可能对治疗有益。
