Imashuku S, Hibi S, Tabata Y, Itoh E, Hashida T, Tsunamoto K, Ishimoto K, Kawano F
Division of Pediatrics, Children's Research Hospital, Kyoto Prefectural University of Medicine Kyoto, Japan.
Leuk Lymphoma. 2000 May;37(5-6):577-84. doi: 10.3109/10428190009058510.
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.
我们通过对爱泼斯坦-巴尔病毒基因组末端、T细胞受体基因和克隆性染色体异常的分析来确定克隆性，研究其对32例噬血细胞性淋巴组织细胞增生症（HLH）患者临床结局的影响。在所研究的病例中，23例为EBV克隆性，15例为TCR克隆性，7例为细胞遗传学克隆性。30例患者接受了免疫化疗和/或多药化疗，4例接受了骨髓移植。所有7例鉴定出细胞遗传学异常克隆的病例均死亡（通过Kaplan-Meier分析的3年生存率为14%，95%置信区间：0-40%）。这7例患者均未接受骨髓移植。另一方面，23例克隆性EBV阳性HLH病例（包括4例细胞遗传学异常病例）的3年生存率为64%（95%置信区间：42-84%），而15例TCR克隆性病例的3年生存率为53%（95%置信区间：26-78%）。我们的观察结果表明，细胞遗传学异常的病例处于极高风险中，需要强化免疫化疗，随后及时进行异基因骨髓移植。
