Fatal haemophagocytic syndrome

S A Fadilah1, A A Raymond, S K Cheong

  • 1Division of Clinical Haematology and Stem Cell Transplantation, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur.

A fulminant clinical presentation with high fever and hepatosplenomegaly, together with a course of worsening pancytopenia, coagulopathy and liver failure, is suggestive of the haem syndrome (HPS). Bone marrow examination is diagnostic. We present 3 cases of HPS associated with different aetiologies including acute Ebstein Barr virus infection, T cell lymphoma, and malignant histiocytosis. In all the cases, the diagnosis was made late and the patients succumbed before definitive therapy could be administered.

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