Abstract:
:Haemophagocytic lymphohistiocytosis secondary to viral infection is an unusual but well recognised cause of bone marrow dysfunction and multiple organ failure in young patients. Two 18 year-old patients were admitted to a tertiary liver unit with features of acute liver failure, cardio-respiratory collapse and pancytopenia. Serological tests and bone marrow examination with in-situ hybridisation revealed severe acquired haemophagocytic lymphohistiocytosis secondary to acute Epstein-Barr virus infection. Both patients died despite full supportive therapy; the first due to pulmonary haemorrhage, the second due to acute respiratory distress syndrome refractory to high frequency oscillatory ventilation. The clinical spectrum, diagnostic features and current evidence based recommendations for treatment of this condition are explored. The diagnosis of haemophagocytic lymphohistiocytosis should be considered in young patients with marked bone marrow dysfunction and multiple organ failure. Further research into appropriate therapy for patients with acute severe forms of the disease who require intensive organ support is required.
journal_name
Anaesthesiajournal_title
Anaesthesiaauthors
Berry PA,Bernal W,Pagliuca A,Sizer E,Salisbury JR,Wendon JA,Auzinger Gdoi
10.1111/j.1365-2044.2008.05584.xsubject
Has Abstractpub_date
2008-11-01 00:00:00pages
1249-54issue
11eissn
0003-2409issn
1365-2044pii
ANA5584journal_volume
63pub_type
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