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Haemophagocytic lymphohistiocytosis after heart transplantation: a case report.
Eur Heart J Case Rep. 2020 Jun; 4(3):1-4.EH

Abstract

Background

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.

Case summary

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.

Discussion

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.

Authors+Show Affiliations

Department of Transplant Institute, Sahlgrenska University Hospital, SE-413 25, Gothenburg, Sweden.Department of Transplant Institute, Sahlgrenska University Hospital, SE-413 25, Gothenburg, Sweden. Department of Cardiology, Sahlgrenska University Hospital, SE-413 25, Gothenburg, Sweden.Department of Transplant Institute, Sahlgrenska University Hospital, SE-413 25, Gothenburg, Sweden. Department of Cardiothoracic Surgery, Sahlgrenska University Hospital, SE-413 25, Gothenburg, Sweden. Department of Molecular and Clinical Medicine, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, SE-413 90 Gothenburg, Sweden.

Pub Type(s)

Case Reports

Language

eng

PubMed ID

32617508

Citation

Danielsson, Christian, et al. "Haemophagocytic Lymphohistiocytosis After Heart Transplantation: a Case Report." European Heart Journal. Case Reports, vol. 4, no. 3, 2020, pp. 1-4.
Danielsson C, Karason K, Dellgren G. Haemophagocytic lymphohistiocytosis after heart transplantation: a case report. Eur Heart J Case Rep. 2020;4(3):1-4.
Danielsson, C., Karason, K., & Dellgren, G. (2020). Haemophagocytic lymphohistiocytosis after heart transplantation: a case report. European Heart Journal. Case Reports, 4(3), 1-4. https://doi.org/10.1093/ehjcr/ytaa070
Danielsson C, Karason K, Dellgren G. Haemophagocytic Lymphohistiocytosis After Heart Transplantation: a Case Report. Eur Heart J Case Rep. 2020;4(3):1-4. PubMed PMID: 32617508.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Haemophagocytic lymphohistiocytosis after heart transplantation: a case report. AU - Danielsson,Christian, AU - Karason,Kristjan, AU - Dellgren,Göran, Y1 - 2020/05/03/ PY - 2019/09/20/received PY - 2019/10/29/revised PY - 2020/03/12/accepted PY - 2020/7/4/entrez PY - 2020/7/4/pubmed PY - 2020/7/4/medline KW - Case report KW - Haemophagocytic Lymphohistiocytosis KW - Haemophagocytic syndrome KW - Heart transplantation SP - 1 EP - 4 JF - European heart journal. Case reports JO - Eur Heart J Case Rep VL - 4 IS - 3 N2 - Background: 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. Case summary: 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. Discussion: 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. SN - 2514-2119 UR - https://www.unboundmedicine.com/medline/citation/32617508/Haemophagocytic_lymphohistiocytosis_after_heart_transplantation:_a_case_report DB - PRIME DP - Unbound Medicine ER -
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