Haemophagocytic syndrome and rickettsial diseases. A new case and systematic review of the literature

Haemophagocytic lymphohistiocytosis is a rare but potentially fatal disease resulting fromdysregulated activation and proliferation of lymphocytes. We present a case of haemophagocyticsyndrome occurring in a 5-year-old Italian boy as a complication of Mediterranean spotted fever.The characteristics of this case have been analysed and contextualized among those of another15 cases of haemophagocytic syndrome associated with rickettsial diseases found through asystematic review of the international literature.