Fatal Haemophagocytic Syndrome

Haemophagocytosis is a phenomenon seen in cytology specimens or tissue sections where histiocytes are seen to engulf a variety of haemopoietic cells. Haemophagocytic syndrome (HPS) presents with fever, pancytopenia, liver dysfunction and increase in haemophagocytic histiocytes in various organs. There are 2 major clinical entities related to the HPS: the aggressive disease known as malignant histiocytosis and the more benign, reactive BPS such as virus-associated HPS • It may be difficult to distinguish malignant BPS from reactive HPS by the clinical course and laboratory data. Early and accurate diagnosis is vital to enhance the chance of success of treatment. This report highlights the need to recognise HPS so that early diagnosis of the associated conditions can be made.