Danielsson Christian, Karason Kristjan, Dellgren Göran
Department of Transplant Institute, Sahlgrenska University Hospital, SE-413 25, Gothenburg, Sweden.
Department of Cardiology, Sahlgrenska University Hospital, SE-413 25, Gothenburg, Sweden.
Eur Heart J Case Rep. 2020 May 3;4(3):1-4. doi: 10.1093/ehjcr/ytaa070. eCollection 2020 Jun.
Haemophagocytic lymphohistiocytosis (HLH) is an uncommon but serious systemic inflammatory response with high mortality rates. It can be triggered by malignancy or infectious agents, often in the context of immunosuppression. Literature covering HLH in heart transplantation (HTx) is scarce.
A 25-year-old male with a history of celiac disease underwent HTx at Sahlgrenska Hospital in 2011 due to giant cell myocarditis and was treated with tacrolimus, mycophenolate mofetil (MMF), and prednisolone. He developed several episodes of acute cellular rejections (ACR) during the first 3 post-HTx years, which subsided after addition of everolimus. In May 2017, the patient was admitted to the hospital due to fever without focal symptoms. He had an extensive inflammatory reaction, but screening for infectious agents was negative. Haemophagocytic lymphohistiocytosis was discussed early, but first dismissed since two bone marrow biopsies revealed no signs of haemophagocytosis. Increasing levels of soluble IL-2 were considered confirmative of the diagnosis. Even with intense immunosuppressant treatment, the patient deteriorated and died in progressive multiorgan failure within 2 weeks of the symptom onset.
A 25-year-old HTx recipient with an extensive inflammatory response, fulfilled criteria for HLH, but the diagnosis was delayed due to normal bone marrow biopsies. A background with autoimmune reactivity and immunosuppressive therapy may have contributed to HLH, but the actual trigger was not identified. Haemophagocytic lymphohistiocytosis can occur in HTx recipients in the absence of malignancy, identifiable infectious triggers and signs of haemophagocytosis. Early diagnosis and intervention are likely to be of importance for a favourable outcome.
噬血细胞性淋巴组织细胞增生症（HLH）是一种罕见但严重的全身炎症反应，死亡率很高。它可由恶性肿瘤或感染因子引发，通常发生在免疫抑制的情况下。关于心脏移植（HTx）中HLH的文献很少。
一名25岁患有乳糜泻病史的男性，因巨细胞性心肌炎于2011年在萨尔格伦斯卡医院接受心脏移植，接受了他克莫司、霉酚酸酯（MMF）和泼尼松龙治疗。在心脏移植后的前3年中，他发生了几次急性细胞排斥反应（ACR），在加用依维莫司后症状缓解。2017年5月，该患者因发热但无局部症状入院。他有广泛的炎症反应，但感染因子筛查为阴性。早期讨论了噬血细胞性淋巴组织细胞增生症，但最初被排除，因为两次骨髓活检均未发现噬血细胞现象。可溶性白细胞介素-2水平升高被认为是确诊的依据。即使进行了强化免疫抑制治疗，患者仍病情恶化，在症状出现后2周内死于进行性多器官功能衰竭。
一名25岁的心脏移植受者出现广泛的炎症反应，符合HLH标准，但由于骨髓活检正常，诊断被延迟。自身免疫反应背景和免疫抑制治疗可能促成了HLH，但实际触发因素未明确。噬血细胞性淋巴组织细胞增生症可发生在心脏移植受者中，而不存在恶性肿瘤、可识别的感染触发因素和噬血细胞现象。早期诊断和干预可能对良好的预后很重要。
