Haemophagocytic lymphohistiocytosis, interferon-gamma-naemia and Epstein-Barr virus involvement

• Published in: British journal of haematology Vol. 88; no. 3; p. 656
• Main Authors: Imashuku, S, Hibi, S, Fujiwara, F, Ikushima, S, Todo, S
• Format: Journal Article
• Language: English
• Published: England 01-11-1994
• Subjects: Adolescent; Child; DNA, Viral - analysis; Female; Herpesvirus 4, Human - genetics; Herpesvirus 4, Human - isolation & purification; Histiocytosis, Non-Langerhans-Cell - blood; Histiocytosis, Non-Langerhans-Cell - mortality; Histiocytosis, Non-Langerhans-Cell - virology; Humans; Infant; Interferon-gamma - blood; Male; Middle Aged; Prognosis
• ISSN: 0007-1048
• DOI: 10.1111/j.1365-2141.1994.tb05095.x

To clarify the correlation between Epstein-Barr virus (EBV) involvement and hypercytokinaemia in haemophagocytic lymphohistiocytosis (HLH), we analysed serum interferon-gamma levels and EBV-DNA in biological specimens obtained from 25 HLH cases (23 children and two adults). We found that HLH patients showed a wide range of serum IFN-gamma levels from 0.2 to 1300 U/ml, with a median 126 U/ml for EBV-DNA-positive (n = 9) and 4.5 U/ml for EBV-DNA-negative (n = 16) groups. The latter group could be classified further into a group with hyper-IFN-gamma-naemia (> 4.5 U/ml) (n = 8) and a group without hyper-IFN-gamma-naemia (n = 8). The survival of the hyper-IFN-gamma-naemic cases was significantly poorer than non-hyper-IFN-gamma-naemic cases. We conclude that EBV is probably involved in one third of the HLH cases, all of whom show hyper-IFN-gamma-naemia, and in the half of the HLH cases with hyper-IFN-gamma-naemia who have a rapidly fatal outcome.