Successful Treatment of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis with HLH-94 Protocol.
10.3346/jkms.2005.20.2.209
- Author:
Jin Seok LEE
1
;
Jin Han KANG
;
Geon Kook LEE
;
Hyeon Jin PARK
Author Information
1. Department of Pediatrics, College of Medicine, Chungbuk National University, Cheongju, Korea. hjpark@chungbuk.ac.kr
- Publication Type:Original Article ; Research Support, Non-U.S. Gov't
- Keywords:
Epstein-Barr Virus Infections;
Hemophagocytic Lymphohistiocytosis;
Histiocytosis, Non-Langer-hans-Cell;
Etoposide;
Cyclosporine;
HLH-94;
Child
- MeSH:
Adolescent;
Bone Marrow Transplantation;
Child, Preschool;
Cyclosporine/administration & dosage;
Dexamethasone/administration & dosage;
Drug Therapy, Combination;
Epstein-Barr Virus Infections/*drug therapy;
Etoposide/administration & dosage;
Female;
Histiocytosis, Non-Langerhans-Cell/*drug therapy;
Humans;
Male;
Research Support, Non-U.S. Gov't
- From:Journal of Korean Medical Science
2005;20(2):209-214
- CountryRepublic of Korea
- Language:English
-
Abstract:
Hemophagocytic lymphohistiocytosis (HLH) is a rare, fatal disorder of children, affecting predominantly the mononuclear phagocytic system. Previous reports indicate that Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (EBV-HLH) can also be fatal in many cases, although the prognosis for EBV-HLH is better than for the familial form of hemophagocytic lymphohistiocytosis. We treated four patients with EBV-HLH using immunochemotherapy including steroid, etoposide (VP-16), and cyclosporin, according to the HLH-94 protocol. All patients experienced persistent fever, cytopenia, and hypertriglyceridemia. Serological testing for EBV showed reactivated EBV infections in all patients. EBV DNA detected by PCR and EBV-encoded small RNA measured by in situ hybridization were confirmed in the patients' bone marrow specimens. Hemophagocytosis was shown in bone marrow aspirates and liver biopsy specimen. Complete remission was achieved in all patients after induction and continuation therapy for 4-10 months (median, 7 months) and was maintained for 15-27 months (median, 19 months) without the need for bone marrow transplantation. These results suggest that EBV-HLH can be effectively controlled by immunochemotherapy using the HLH-94 protocol.
