Haemophagocytic syndrome and rickettsial diseases

Haemophagocytic lymphohistiocytosis is a rare but potentially fatal disease resulting from dysregulated activation and proliferation of lymphocytes. We present a case of haemophagocytic syndrome 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 another 15 cases of haemophagocytic syndrome associated with rickettsial diseases found through a systematic review of the international literature.