Successful Treatment of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis with HLH-94 Protocol
Journal of Korean Medical Science
;
: 209-214, 2005.
Article
Dans Anglais
| WPRIM
| ID: wpr-8398
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.
Texte intégral:
Disponible
Indice:
WPRIM (Pacifique occidental)
Sujet Principal:
Dexaméthasone
/
Histiocytose non langerhansienne
/
Transplantation de moelle osseuse
/
Ciclosporine
/
Infections à virus Epstein-Barr
/
Association de médicaments
/
Étoposide
Type d'étude:
Guide de pratique
Limites du sujet:
Adolescent
/
Enfant d'âge préscolaire
/
Femelle
/
Humains
/
Mâle
langue:
Anglais
Texte intégral:
Journal of Korean Medical Science
Année:
2005
Type:
Article
Documents relatifs à ce sujet
MEDLINE
...
LILACS
LIS