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首页> 外文期刊>Clinical kidney journal. >Rejection-triggered haemophagocytic syndrome in renal transplantation successfully treated with intravenous immunoglobulin
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Rejection-triggered haemophagocytic syndrome in renal transplantation successfully treated with intravenous immunoglobulin

机译：静脉免疫球蛋白成功治疗肾移植排斥反应引发的噬血细胞综合征

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Haemophagocytic syndrome (HPS) is a rare and potentially lethal condition characterized by pancytopoenia, fever, organomegaly and widespread proliferation of macrophages phagocytosing blood elements. Among the triggers of this syndrome, excessive immunosuppression in a context of acute rejection has been rarely reported, although it might be underdiagnosed. Here, we report the case of a kidney transplant recipient with allograft dysfunction due to chronic antibody-mediated rejection treated with antithymocyte globulin and plasmapheresis. The patient developed high fever, pancytopoenia, diarrhoea and respiratory symptoms with no apparent infectious or neoplastic cause, despite an extensive work-up. Haemophagocytosis was found in bone marrow examination, along with hyperferritinaemia and hypertriglyceridaemia. The clinical profile improved after treatment with intravenous immunoglobulin and reduction of the basal immunosuppression.
机译：噬血细胞综合征（HPS）是一种罕见的潜在致命疾病，其特征是全血细胞减少，发热，器官肿大和巨噬细胞吞噬血液元素的广泛扩散。在该综合征的触发因素中，尽管可能未得到充分诊断，但很少有关于急性排斥反应中过度免疫抑制的报道。在这里，我们报道了由于用抗胸腺细胞球蛋白和血浆置换治疗慢性抗体介导的排斥反应而导致的同种异体移植功能障碍的肾脏移植受者的情况。尽管进行了广泛的检查，但患者仍出现高烧，全血细胞减少症，腹泻和呼吸道症状，没有明显的感染或赘生性病因。在骨髓检查中发现了噬血细胞作用，并伴有高铁蛋白血症和高甘油三酸酯血症。静脉注射免疫球蛋白治疗和减少基础免疫抑制后，临床情况有所改善。

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