EBV and hemophagocytic syndrome: analysis of 3 cases, with speculation on clinical features, therapy and role of EBV

Abstract

The authors report three patients diagnosed with EBV associated HPCS. The first case died of a fatal EBV infection. The second and third cases had primary disease of malignant lymphoma. In case 2, T cell lymphoma associated HPCS was diagnosed early. However, despite the aggressive treatment of HPCS and T-cell lymphoma, the patient died because of the refractory lymphoma. In case 3, HPCS and B malignant lymphoma were diagnosed at post mortem. EBV was found very late in all three cases. Case 1 and case 2 had a very high DNA-EBV load in blood. Case 3 demonstrated EBV-RNA encoded antigen (EBER) in lymph node by in situ hybridization technique. The clinical features of HPCS were analysed. Four early manifestations in these three cases were emphasized, namely fever, splenomegaly, progressive pancytopenia and impaired liver function test without severe jaundice. The authors stress the most important factor to save the life of the patients is to give early diagnosis and early proper management of HPCS as well as the etiologic diseases. The treatment of choice of early HPCS are pulse corticosteroid, IVIgG. Combination immunochemotherapy including pulse corsticosteroid, IVIgG, cyclosporin A, etoposide and plasma exchange should be given promptly in severe cases. From the present report, it indicates that the association of EBV with HPCS is not uncommon in Thailand. EBV is very important because it gives a very poor prognosis either by being an etiologic cause of HPCS or by association with ML with HPCS. Clinicians should be aware of EBV and recognize it early. The early treatment of EBV should helpfully changes the prognosis of the patients. The role of EBV on the occurrence of HPCS and T-ML is also discussed