Fatal haemophagocytic syndrome.
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.
Fadilah
S A
SA
Division of Clinical Haematology and Stem Cell Transplantation, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur.
Raymond
A A
AA
Cheong
S K
SK
Amir
M A
MA
D000328:Adult; D017809:Fatal Outcome; D015616:Histiocytosis, Non-Langerhans-Cell / Q000401:mortality* / Q000473:pathology* / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged