Haemophagocytic lymphohistiocytosis in kidney transplant recipients

Abstract

Hemophagocytic lymphohistiocytosis (HLH) in renal transplant recipients is a life-threatening hyper-inflammatory syndrome; associated with uncontrolled activation of cytotoxic T-lymphocytes and macrophages due to infections or immunosuppressive therapy. Histoplasmosis, tuberculosis and herpes virus infection are among the leading infectious causes. It is characterized by fever, organomegaly, cytopenia, hyperferritinemia, hypertrigiceridemia and/or hypofibrinogenemia; which may be accompanied by hemophagocytosis in bone marrow, liver or other organs. LH can follow a rapidly fatal course, with progression to multisystemic failure and death. The treatment is based on early control of the triggering cause, reducing immunosuppression and stop the inflammatory process. In some cases, is necessary to use other immunosuppressant, chemotherapy and in a very few cases, a bone marrow transplant may be required.

Keywords

• hemophagocytic lymphohistiocytosis
• histoplasmosis
• ferritins
• kidney transplantation