Diagnostic challenge of recurrent macrophage activation syndrome before and after kidney transplant

摘要

Hemophagocytic lymphohistiocytosis (HLH) is a rare, life- threatening condition, secondary to an overwhelming inflam- matory process. When associated to rheumatic disorders, it can be called as macrophage activation syndrome. 1 The main manifestations are unremitting fever, cytopenias, hepatosplenomegaly and multisystem organ failure. Unfortu- nately, there is no pathognomonic test, making the diagnosis hard to reach. 2 It can be triggered by infection, malignancy, auto-inflammatory disease and immunosuppression asso- ciated with solid organ transplantation. Kidney transplant recipients are, particularly, at risk, and in most cases, infection is the identified trigger.