Longitudinal observation and outcome of nonfamilial childhood haemophagocytic syndrome receiving etoposide-containing regimens
Journal
British Journal of Haematology
Journal Volume
103
Journal Issue
3
Pages
756-762
Date Issued
1998
Author(s)
Abstract
The long-term outcome of 22 children treated with etoposide-containing regimens for haemophagocytic syndrome (HS) were longitudinally studied; none of them had a family history of the disease. All patients received etoposide- containing (150mg/m2/d) regimens, combined, in 16 cases, with intravenous immunoglobulin (IVIG) and prednisolone. Complete remission (CR) was achieved in 12 patients, partial remission in seven, and early mortality occurred in three. Of the 12 CR patients, only four remain alive and disease-free, with a median follow-up of 47.4 months; one CR patient died due to infection and the remaining seven had relapsed diseases. Three patients with a partial response or with relapsed disease progressed to T-cell lymphoma, characterized, in the two cases tested, by clonal chromosomal abnormalities. Epstein-Barr virus (EBV) infection was implicated in disease pathogenesis in 15/22 patients. The overall survival was 45.5%, 40.9% and 40.9% at 1, 3 and 5 years, respectively, and disease-free survival for CR patients at these same times was 45.5%, 36.4% and 36.4%. The etoposide-containing regimen would appear to be an effective initial therapeutic option for childhood HS. However, in view of the frequency of partial remissions and relapsed disease, a more intensive chemotherapy or bone marrow transplantation should be applied. The progression to EBV-containing T-cell lymphoma in three patients is consistent with the previous observation that EBV-associated HS is a potentially malignant disease.
Subjects
Epstein-Barr virus; Etoposide; Haemophagocytic syndrome; Survival; T-cell lymphoma
SDGs
Other Subjects
aciclovir; etoposide; ganciclovir; immunoglobulin; immunoglobulin g; immunoglobulin m; prednisolone; virus capsid antigen; adolescent; article; child; childhood disease; clinical article; clinical trial; epstein barr virus; female; hemophagocytic syndrome; human; infant; intravenous drug administration; longitudinal study; male; mortality; observation; priority journal; remission; t cell lymphoma; treatment outcome; virus infection; Adolescent; Antineoplastic Agents, Hormonal; Antineoplastic Agents, Phytogenic; Antineoplastic Combined Chemotherapy Protocols; Child; Child, Preschool; Disease-Free Survival; Etoposide; Female; Herpesviridae Infections; Histiocytosis, Non-Langerhans-Cell; Humans; Infant; Karyotyping; Longitudinal Studies; Male; Prednisolone; RNA, Viral; Treatment Outcome
Type
journal article