title: Outcome of clonal hemophagocytic lymphohistiocytosis: analysis of 32 cases.
abstract: 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.
authors.affiliation: Division of Pediatrics, Children's Research Hospital, Kyoto Prefectural University of Medicine Kyoto, Japan. shinik95@mbox.kyoto-inet.or.jp
authors.forename: S
authors.initials: S
authors.lastname: Imashuku
authors.forename: S
authors.initials: S
authors.lastname: Hibi
authors.forename: Y
authors.initials: Y
authors.lastname: Tabata
authors.forename: E
authors.initials: E
authors.lastname: Itoh
authors.forename: T
authors.initials: T
authors.lastname: Hashida
authors.forename: K
authors.initials: K
authors.lastname: Tsunamoto
authors.forename: K
authors.initials: K
authors.lastname: Ishimoto
authors.forename: F
authors.initials: F
authors.lastname: Kawano
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000782:Aneuploidy; D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D002999:Clone Cells / Q000473:pathology; D003131:Combined Modality Therapy; D016572:Cyclosporine / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D020031:Epstein-Barr Virus Infections / Q000175:diagnosis; D005047:Etoposide / Q000627:therapeutic use; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D015616:Histiocytosis, Non-Langerhans-Cell / Q000401:mortality* / Q000473:pathology / Q000628:therapy / Q000821:virology; D006801:Humans; D007223:Infant; D007564:Japan / Q000453:epidemiology; D008297:Male; D011239:Prednisolone / Q000627:therapeutic use; D011379:Prognosis; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D005047:Etoposide; D016572:Cyclosporine; D011239:Prednisolone