Haemophagocytic lymphohistiocytosis, interferon-gamma-naemia and Epstein-Barr virus involvement.
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.
Imashuku
S
S
Division of Paediatrics, Children's Research Hospital, Kyoto Prefectural University of Medicine, Japan.
Hibi
S
S
Fujiwara
F
F
Ikushima
S
S
Todo
S
S
D000293:Adolescent; D002648:Child; D004279:DNA, Viral / Q000032:analysis; D005260:Female; D004854:Herpesvirus 4, Human / Q000235:genetics / Q000302:isolation & purification*; D015616:Histiocytosis, Non-Langerhans-Cell / Q000097:blood* / Q000401:mortality / Q000821:virology; D006801:Humans; D007223:Infant; D007371:Interferon-gamma / Q000097:blood*; D008297:Male; D008875:Middle Aged; D011379:Prognosis
D004279:DNA, Viral; D007371:Interferon-gamma