Osband M E, Lipton J M, Lavin P, Levey R, Vawter G, Greenberger J S, McCaffrey R P, Parkman R
N Engl J Med. 1981 Jan 15;304(3):146-53. doi: 10.1056/NEJM198101153040304.
Twelve of 17 patients with histiocytosis-X were immunologically abnormal, as shown by the presence of circulating lymphocytes spontaneously cytotoxic to cultured human fibroblasts or of antibody to autologous erythrocytes. The patients also had a notable lack of histamine H2 surface receptors on their T lymphocytes, suggesting a suppressor-cell deficiency. The lymphocyte abnormalities were reversed in vitro after incubation in a crude extract of calf thymus gland, and therefore all 17 patients were treated with daily intramuscular injections of this extract. With this therapy, 10 patients entered complete remission -- a response at least as good as that observed in historical controls treated with chemotherapy. A positive clinical response was associated with an increase in the number of T-cell histamine H2 receptors to normal levels and with correction of the other immunologic abnormalities. The results of this preliminary study justify a larger prospective clinical trial of thymic extract and further investigation of the immunoregulatory mechanisms in histiocytosis-X.
17例组织细胞增多症-X患者中有12例存在免疫异常，表现为循环淋巴细胞对培养的人成纤维细胞具有自发细胞毒性，或存在抗自体红细胞抗体。这些患者的T淋巴细胞上还明显缺乏组胺H2表面受体，提示存在抑制细胞缺陷。在小牛胸腺粗提物中孵育后，淋巴细胞异常在体外得到逆转，因此对所有17例患者每日进行该提取物的肌肉注射治疗。通过这种治疗，10例患者实现了完全缓解——这一反应至少与化疗的历史对照观察结果一样好。临床阳性反应与T细胞组胺H2受体数量增加至正常水平以及其他免疫异常的纠正有关。这项初步研究的结果证明有必要对胸腺提取物进行更大规模的前瞻性临床试验，并进一步研究组织细胞增多症-X中的免疫调节机制。
