Outcome of Clonal Hemophagocytic Lymphohistiocytosis: Analysis of 32 Cases

We studied the impact of clonality, determined by analysis of Epstein-Barr virus genome termini, T-cell receptor genes and clonal chromosomal abnormality, on the clinical outcome in 32 patients with hemophagocytic lymphohistiocytosis (HLH). Of the cases studied, 23 cases were EBV-clonal, 15 cases we...

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Bibliographic Details
Published in:Leukemia & lymphoma 2000, Vol.37 (5-6), p.577-584
Main Authors: Imashuku, Shinsaku, Hibi, Shigeyoshi, Tabata, Yasuhiro, Itoh, Etsuro, Hashida, Tetsuo, Tsunamoto, Kentaro, Ishimoto, Koichi, Kawano, Fumio
Format: Article
Language:English
Subjects:
Adolescent
Adrenal Cortex Hormones - therapeutic use
Adult
Aneuploidy
Bone Marrow Transplantation
Child
Child, Preschool
chromosomal abnormality
clonality
Clone Cells - pathology
Combined Modality Therapy
Cyclosporine - therapeutic use
Drug Therapy, Combination
Epstein-Barr virus infection
Epstein-Barr Virus Infections - diagnosis
Etoposide - therapeutic use
Female
Gene Rearrangement, T-Lymphocyte
hemophagocytic lymphohistiocytosis
Herpesvirus 4, Human - isolation & purification
Histiocytosis, Non-Langerhans-Cell - mortality
Histiocytosis, Non-Langerhans-Cell - pathology
Histiocytosis, Non-Langerhans-Cell - therapy
Histiocytosis, Non-Langerhans-Cell - virology
Humans
Infant
Japan - epidemiology
Male
Prednisolone - therapeutic use
Prognosis
Treatment Outcome
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Summary:We studied the impact of clonality, determined by analysis of Epstein-Barr virus genome termini, T-cell receptor genes and clonal chromosomal abnormality, on the clinical outcome in 32 patients with hemophagocytic lymphohistiocytosis (HLH). Of the cases studied, 23 cases were EBV-clonal, 15 cases were TCR-clonal and 7 cases were cytogenetically clonal. Thirty patients were treated with immuno-chemotherapy and/or multiagents' chemotherapy and 4 received bone marrow transplantation. All 7 cases, in which cytogenetically abnormal clones were identified, were fatal (3-year survival by Kaplan-Meier analysis; 14%, 95%CI 0-40%). None of these 7 cases received bone marrow transplantation. On the other hand, the 3-year survival of 23 clonal EBV-positive HLH cases including 4 cytogenetically abnormal cases was 64 % (95%CI: 42-84%), while that of 15 TCR-clonal cases was 53% (95%CI: 26-78%). Our observations suggest that cytogenetically abnormal cases are at extremely high risk, requiring intensive immuno-chemotherapy followed by prompt and timely allogeneic bone marrow transplantation.
ISSN:1042-8194
1029-2403
DOI:10.3109/10428190009058510