Watanaboonyongcharoen Phandee, Whinna Herbert C, Park Yara A
Department of Pathology and Laboratory Medicine, University of North Carolina, Chapel Hill, North Carolina; Transfusion Medicine Unit, King Chulalongkorn Memorial Hospital, Bangkok, Thailand.
J Clin Apher. 2014 Dec;29(6):336-8. doi: 10.1002/jca.21322. Epub 2014 Apr 25.
Idiopathic thrombotic thrombocytopenic purpura (TTP) patients have ADAMTS13 deficiency, which is usually caused by ADAMTS13 autoantibodies. However, the triggering factors for the autoantibody production remain unclear. Interferon-α (IFN-α) is a cytokine involved with many autoimmune processes such as inducing the activation of peripheral dendritic cells and stimulating T cells and B cells. It also plays an important role in some autoimmune diseases. Elevated IFN-α levels have been observed in some TTP patients and previous case reports have shown the occurrence of TTP after IFN-α treatment. Thus, we hypothesized that high levels of IFN-α would correlate with presence of ADAMTS13 autoantibodies. However, we did not observe elevated IFN-α levels in 36 TTP patients (mean 5.29 pg/ml, standard deviation (SD) 26.56 pg/ml) compared to healthy controls (mean 0 pg/ml, SD 0 pg/ml), P = 0.59. IFN-α levels of most patients (94%) were undetectable. Only two patients had increased IFN-α levels and ADAMTS13 autoantibodies were detected in these two patients. Interestingly, both the patients had an underlying autoimmune disease. Although there have been cases of secondary TTP following IFN-α treatment, no evidence supports a role of IFN-α in the development of idiopathic TTP in our patient population.
特发性血栓性血小板减少性紫癜（TTP）患者存在ADAMTS13缺乏，这通常由ADAMTS13自身抗体引起。然而，自身抗体产生的触发因素仍不清楚。干扰素-α（IFN-α）是一种细胞因子，参与许多自身免疫过程，如诱导外周树突状细胞活化以及刺激T细胞和B细胞。它在一些自身免疫性疾病中也起重要作用。在一些TTP患者中观察到IFN-α水平升高，并且既往病例报告显示IFN-α治疗后发生了TTP。因此，我们推测高水平的IFN-α与ADAMTS13自身抗体的存在相关。然而，与健康对照（平均0 pg/ml，标准差（SD）0 pg/ml）相比，我们在36例TTP患者中未观察到IFN-α水平升高（平均5.29 pg/ml，SD 26.56 pg/ml），P = 0.59。大多数患者（94%）的IFN-α水平无法检测到。只有两名患者的IFN-α水平升高，且在这两名患者中检测到了ADAMTS13自身抗体。有趣的是，这两名患者均患有潜在的自身免疫性疾病。虽然有IFN-α治疗后发生继发性TTP的病例，但在我们的患者群体中，没有证据支持IFN-α在特发性TTP发病中的作用。
