IMMUNOVIROLOGICAL STUDIES OF FATAL INFECTIOUS-MONONUCLEOSIS IN A PATIENT WITH X-LINKED LYMPHOPROLIFERATIVE SYNDROME TREATED WITH INTRAVENOUS IMMUNOGLOBULIN AND INTERFERON-ALPHA
IMMUNOVIROLOGICAL STUDIES OF FATAL INFECTIOUS-MONONUCLEOSIS IN A PATIENT WITH X-LINKED LYMPHOPROLIFERATIVE SYNDROME TREATED WITH INTRAVENOUS IMMUNOGLOBULIN AND INTERFERON-ALPHA
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DOI：
10.1016/0090-1229(90)90054-t
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发表时间：
1990-03-01
期刊：
影响因子：
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通讯作者：
PURTILO, DT
中科院分区：
文献类型：
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作者：
OKANO, M;PIRRUCCELLO, SJ;PURTILO, DT
我们研究了一名患有X连锁淋巴增殖综合征（XLP）和传染性单核细胞增多症（IM）的19岁男性，他接受了高剂量免疫球蛋白（500毫克/千克/天）和重组干扰素（IFN）-α（2×10⁶国际单位/平方米/天）的治疗。暴发性肝炎被延迟；然而，最终出现了病毒相关的噬血细胞综合征、胆汁淤积性黄疸和肾衰竭。最初，针对K562细胞的非特异性自然杀伤（NK）细胞活性正常，但逐渐下降。尽管在急性期他血液中的反应性T细胞显著增加，但很容易建立自发性EB病毒阳性细胞系。此外，在治疗前他的单核细胞产生IFN -γ，但不产生IFN -α。基于体外研究的结果，我们得出结论，IFN -α和IFN -γ的产生可能对于在体外抑制EB病毒永生化都是必要的。在来自EB病毒血清阳性健康个体的B95 - 8 EB病毒感染的单核细胞培养物中，IFN -α和 -γ都被产生。这些结果表明，EB病毒特异性细胞毒性T细胞活性缺陷，伴随着IFN -α和 -γ产生缺陷或不协调，导致了该患者致命性IM的发生。在这种疾病的早期阶段，免疫球蛋白和IFN -α的联合治疗似乎部分有效。
We have studied a 19-year-old male with X-linked lymphoproliferative syndrome (XLP) and infectious mononucleosis (IM) who was treated with high-dose immunoglobulin (500 mg/kg/day) and recombinant interferon (IFN)-.alpha. (2 .times. 106 IU/m2/day). Fulminant hepatitis was delayed; however, virus-associated hemophagocytic syndrome, cholestatic jaundice, and renal failure occurred terminally. Initially, nonspecific natural killer (NK) cell activity against K562 cells was normal but it gradually decreased. Although reactive T cells were markedly increased in his blood during the acute phase, spontaneous EBV-positive cell lines were easily established. Additionally, his mononuclear cells produced IFN-.gamma. but not IFN-.alpha. prior to treatment. Based on results of in vitro studies, we conclude that both IFN-.alpha. and IFN-.gamma. production are likely necessary for inhibiting EBV immortalization in vitro. Both IFN-.alpha. and -.gamma. were produced in cultures of B95-8 EBV-infected mononuclear cells from EBV-seropositive healthy individuals. These results suggest that defective EBV-specific cytotoxic T cell activity accompanied with defective or discordant IFN-.alpha. and -.gamma. production permitted the development of fatal IM in this patient. Combined treatment with immunoglobulin and IFN-.alpha. appeared to be partially effective during the early stage of this disease.