Haemophagocytic syndrome associated to dengue haemorrhagic fever

Abstract

The haemophagocytic syndrome is characterised by systemic proliferation of non-neoplastic histiocytes showing haemophagocytosis resulting in blood cytopaenia. It has been described in relation to several viruses earlier. We present three patients with haemophagocytic syndrome (HFS) secondary to dengue haemorrhagic fever (DHF) confirmed by standard laboratory tests. The patients were hospitalized at the University Hospital (Hospital Universitario Ramon Gonzalez Valencia-HURGV) in Bucaramanga, Colombia, during the past two years. They were all school-aged patients who presented DHF with intense abdominal pain, prolonged fever, hypotension and painful hepatomegaly. Laboratory tests showed thrombocitopenia, anaemia and leucopenia. A calculous cholecystitis was observed in the abdominal ultrasonography, and all bone marrow aspirations showed that platelets, red and white blood cells were phagocyted by histiocites. According to the International Society of Histiocytosis, SHF is defined and classified in three major categories; the reported cases corresponded to histiocytosis class II, specifically to secondary SHF. Diverse associations of this syndrome correspond to viral infections and some other non-infectious diseases. A difference has been established between primary SHF and secondary SHF. Finally, we emphasize that these three patients had an atypical evolution of FHD, being prolonged fever and persistent abdominal pain the most important symptoms.The authors recommend that a bone marrow aspiration should be carried out as part of the differential diagnosis study in prolonged fever associated with dengue, as there is a possibility that this complication could be secondary SHF.