Impaired cell-mediated immunity in patients with classic hemophilia.
Impaired cell-mediated immunity in patients with classic hemophilia.
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典型血友病患者细胞介导的免疫力受损。
DOI：
10.1056/nejm198301133080205
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发表时间：
1983
期刊：
影响因子：
--
通讯作者：
Schacter,B
中科院分区：
文献类型：
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作者：
Lederman,MM;Ratnoff,OD;Scillian,JJ;Jones,PK;Schacter,B
经商业冻干凝血因子VIII治疗的血友病患者现在被认为有可能发展为一种具有获得性免疫缺陷综合征(AIDS)特征的临床疾病。卡氏肺孢子虫肺炎、辅助性T淋巴细胞(T4)相对减少和抑制性T细胞增加在这一组中已被描述。这些观察促使对19名健康的血友病患者和19名年龄匹配的健康对照受试者进行了调查。在前一组中，11人在前一年内接受了3-200次冻干凝血因子VIII浓度的输注(中位数30-36)-LYOPH组；其余8人中有4人接受了冷沉淀物(1人2次补充浓缩物)，4人在前20个月没有接受治疗-冷冻组。与对照组相比，LYOPH组辅助性T细胞相对减少，抑制性T细胞相对和绝对增加，辅助性/抑制性比值降低。然而，与艾滋病患者相比，血友病患者的辅助T4细胞似乎只有相对减少，后者更有可能有绝对的T4细胞减少。LYOPH组还显示自然杀伤细胞活性降低，对有丝分裂原的增殖反应减弱。冷冻组淋巴细胞计数和表面标志与对照组相似。现有证据似乎表明冻干的凝血因子VIII浓缩物中含有某种制剂。乙肝病毒被排除为病因。需要对这一推定代理人的性质进行进一步调查。
Patients with haemophilia treated with commercial lyophilized factor VIII concentrates are now being recognized as at risk of developing a clinical disease with features of acquired immune deficiency syndrome (AIDS).Pneumocystis cariniipneumonia, a relative decrease of helper T lymphocytes (T4) and an increase in suppressor T cells have been described in this group. These observations prompted an investigation of 19 healthy patients with haemophilia and 19 healthy age-matched control subjects. Of the former group 11 had received lyophilized factor VIII concentrates within the previous year ranging from 3 to 200 infusions (median 30-36)-LYOPH group; 4 of the remaining 8 had received cryoprecipitate (1 supplemented on 2 occasions with concentrate) and 4 had received no therapy in the preceding 20 months-CRYO group. When compared with control subjects the LYOPH group had a relative decrease in helper T cells, a relative and absolute increase of suppressor T cells and depressed helper/suppressor ratios. However, it appears that patients with haemophilia had only a relative decrease of helper T4 cells as compared with patients with AIDS who are more likely to have an absolute decrease in T4 cells. The LYOPH group also showed a reduction in natural-killer cell activity and decreased proliferative responses to mitogens. Lymphocyte counts and surface markers in the CRYO group were similar to those in the control group. The available evidence seems to incriminate some agent in lyophilized factor VIII concentrates. Hepatitis B virus was excluded as a cause. Further investigations are required into the nature of this putative agent.K.C. Watson