Rejection-triggered haemophagocytic syndrome in renal transplantation successfully treated with intravenous immunoglobulin

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.