Lepe-Zuniga J L, Quesada J R, Baron S, Reuben J, Gutterman J U
Department of Clinical Immunology and Biological Therapy, University of Texas, Houston 77030.
Hematol Pathol. 1987;1(3):157-65.
Nineteen patients with hairy cell leukemia (HCL) were studied for in vitro production of alpha interferon (IFN alpha). The patients were divided into three groups. The first group consisted of 8 patients with active disease, all of whom showed a severe deficiency in IFN alpha production (no detectable titers in 6 and less than 40 units/ml in the other 2) compared to normal controls (range: 320-10,500 units/ml; median: 2,560 units/ml). The second group consisted of 6 patients who achieved a partial remission (PR) after IFN alpha treatment. These 6 patients had normal numbers of mononuclear cells in the peripheral blood, but still had deficient IFN alpha production (titers of IFN alpha were below 10 units/ml in 5 of the 6 patients). The third group consisted of 5 patients in complete remission (3 after IFN alpha treatment and 1 each after splenectomy and infection). This group had IFN alpha production that was not significantly different from that of controls 35 years or older (median: 640 units/ml; range 60-2,560 units/ml for patients vs. 1,513 units/ml; range 320-5,120 units/ml for controls; p greater than 0.05). These data show a direct relationship between the activity of HCL and the capacity to produce IFN alpha in vitro. The data also suggest that deficiency of endogenous production of IFN alpha may be relevant to the induction and sustenance of remissions in this disease and that relapses may be partly associated with failure to fully restore endogenous IFN alpha production.
对19例毛细胞白血病（HCL）患者进行了α干扰素（IFNα）体外产生情况的研究。患者被分为三组。第一组有8例处于疾病活动期的患者，与正常对照（范围：320 - 10,500单位/毫升；中位数：2,560单位/毫升）相比，所有患者均表现出IFNα产生严重不足（6例检测不到滴度，另外2例低于40单位/毫升）。第二组由6例经IFNα治疗后达到部分缓解（PR）的患者组成。这6例患者外周血单核细胞数量正常，但IFNα产生仍不足（6例患者中有5例IFNα滴度低于10单位/毫升）。第三组由5例完全缓解的患者组成（3例经IFNα治疗后缓解，1例脾切除后缓解，1例感染后缓解）。该组患者的IFNα产生与35岁及以上的对照者相比无显著差异（患者中位数：640单位/毫升；范围60 - 2,560单位/毫升，对照者中位数：1,513单位/毫升；范围320 - 5,120单位/毫升；p＞0.05）。这些数据表明HCL的疾病活动与体外产生IFNα的能力之间存在直接关系。数据还提示，内源性IFNα产生不足可能与该疾病缓解的诱导和维持有关，复发可能部分与内源性IFNα产生未能完全恢复有关。
