BIOMARKER AND MORPHOLOGICAL-CHARACTERISTICS OF EPSTEIN-BARR VIRUS-RELATED HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS

Background. Viruses may induce primary as well as secondary hemophagoc ytic lymphohistiocytosis (HLH), but it may not be possible to discrimi nate between these two in patients with a negative family history. Amo ng these HLH cases, fulminant and fatal virus-associated hemophagocyti c syndrome (VAHS) occurs mostly in relation to Epstein-Barr virus (EBV ) infection. Although the immunological characteristics oi EB-VAHS wer e previously reported, data on non-EB-VAHS were sporadic and fragmenta ry. This study has compared the clearly distinguishable groups of EBV- positive vs. EBV-negative HLH cases. Procedure. Among 26 patients with EBV-related HLH and 12 patients with non-EBV HLH, peripheral blood mo nonuclear cell (PBMC) subsets and serum concentrations of cytokines at the active phase of the disease were compared. Blood and bone marrow smears were also compared. Results and Conclusions. The frequency of t he CD3(+)HLADR(+) subset in PBMC (median 34.3% vs. 4.8%), of serum con centrations of interferon (IFN)-gamma (median 105 U/ml vs. 2.4 U/ml), and of soluble interleukin-2-receptor (sIL-2R) (median 14,700 U/ml vs. 3,412 U/ml) were significantly different between these two groups. Mo rphological characteristics were noted for EBV-related HLH cases. Mort ality also differed between these two groups, 9/26 vs. 0/12 (P = 0.05) . Data indicate pronounced immunological imbalance and poor prognosis in EBV-related HLH cases. These parameters could be useful for determi ning an EBV involvement as well as risk factors in the early care and treatment of HLH patients. Med. Pediatr. Oncol. 31: 131-137, 1998. (C) 1998 Wiley-Liss, Inc.