Multiple organ failure and severe bone marrow dysfunction in two 18 year‐old Caucasian patients: Epstein–Barr virus and the haemophagocytic syndrome

• Published in: Anaesthesia Vol. 63; no. 11; pp. 1249 - 1254
• Main Authors: Berry, P. A., Bernal, W., Pagliuca, A., Sizer, E., Salisbury, J. R., Wendon, J. A., Auzinger, G.
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01.11.2008; Blackwell
• Subjects: Acute Disease; Adolescent; Anesthesia; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Biological and medical sciences; Biopsy; Blood diseases; Bone Marrow Diseases - pathology; Bone Marrow Diseases - virology; Children & youth; Epstein-Barr virus; Epstein-Barr Virus Infections - complications; Fatal Outcome; Female; Humans; Lymphohistiocytosis, Hemophagocytic - diagnosis; Lymphohistiocytosis, Hemophagocytic - pathology; Lymphohistiocytosis, Hemophagocytic - virology; Medical diagnosis; Medical sciences; Medical treatment; Multiple Organ Failure - virology; Viruses; Gammaherpesvirinae; Virus; Human; Hemophagocytic syndrome; Bone marrow failure; Herpesviridae; Multiple organ failure; Anesthesia; Hemopathy; Epstein Barr virus; Histiocytosis
• ISSN: 0003-2409; 1365-2044
• DOI: 10.1111/j.1365-2044.2008.05584.x

Summary 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.