title: Rejection-triggered haemophagocytic syndrome in renal transplantation successfully treated with intravenous immunoglobulin.
abstract: 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.
authors.affiliation: Renal Transplant Service , Hospital das Clínicas, University of São Paulo School of Medicine , São Paulo , Brazil.
authors.forename: Igor Denizarde Bacelar
authors.initials: ID
authors.lastname: Marques
authors.affiliation: Nephrology Division , Hospital das Clínicas, University of São Paulo School of Medicine , São Paulo , Brazil.
authors.forename: Renato Antunes
authors.initials: RA
authors.lastname: Caires
authors.affiliation: Renal Transplant Service , Hospital das Clínicas, University of São Paulo School of Medicine , São Paulo , Brazil.
authors.forename: Flávio Jota
authors.initials: FJ
authors.lastname: de Paula
authors.affiliation: Renal Transplant Service , Hospital das Clínicas, University of São Paulo School of Medicine , São Paulo , Brazil.
authors.forename: William Carlos
authors.initials: WC
authors.lastname: Nahas
authors.affiliation: Renal Transplant Service , Hospital das Clínicas, University of São Paulo School of Medicine , São Paulo , Brazil.
authors.forename: Elias
authors.initials: E
authors.lastname: David-Neto
keywords: allograft rejection; haemophagocytic syndrome; intravenous immunoglobulin; kidney transplantation