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儿童急性淋巴细胞白血病中化疗与骨髓移植的对比。柏林-法兰克福-明斯特(BFM)研究组

Chemotherapy versus bone marrow transplantation in childhood acute lymphoblastic leukaemia. BFM Study Group.

作者信息

Ebell W, Reiter A, Riehm H

机构信息

Department of Paediatric Hematology and Oncology, Children's Hospital, Hannover Medical School, Germany.

出版信息

Eur J Pediatr. 1992;151 Suppl 1:S50-4. doi: 10.1007/BF02125803.

Abstract

Twenty-five years ago over 90% of children with acute lymphoblastic leukaemia (ALL) died of this disease. Dramatic improvement has been achieved since then by employing risk-adapted, aggressive polychemotherapy protocols. More than 90% of children with ALL treated according to, for example BFM-protocols, have nowadays cure rates in the range of 70%-80%. However, 10% of patients do not initially respond adequately to standard induction chemotherapy. They are characterized by distinct chromosomal abnormalities such as translocation (9; 22) or combinations of early treatment failure and other risk factors as cytogenetic abnormalities, lineage-specific surface markers or tumour load at diagnosis. In this group of patients in first complete remission and certainly in the vast majority of relapsed patients, allogeneic bone marrow transplantation (BMT) has evolved as an alternative approach allowing further intensification of myeloablation and the introduction of an additional antileukaemic alloreactivity. Nevertheless, the decision for a marrow transplant in children has to be made very carefully because of a significant increase in treatment related mortality and BMT-specific risks like acute and chronic graft-versus-host disease with a critical iatrogenic chronic morbidity. This is even more evident, if mismatched or unrelated transplants are being considered. The indications for one or the other treatment modality according to the current BFM strategy are discussed.

摘要

25年前,超过90%的急性淋巴细胞白血病(ALL)患儿死于该病。自那时起,通过采用风险适应性、积极的多化疗方案,已取得了显著进展。如今,按照例如BFM方案进行治疗的ALL患儿中,超过90%的治愈率在70%-80%之间。然而,10%的患者最初对标准诱导化疗反应不佳。他们具有明显的染色体异常,如易位(9;22),或早期治疗失败与其他风险因素的组合,如细胞遗传学异常、谱系特异性表面标志物或诊断时的肿瘤负荷。在这组首次完全缓解的患者中,当然也包括绝大多数复发患者,异基因骨髓移植(BMT)已发展成为一种替代方法,可进一步强化清髓,并引入额外的抗白血病同种异体反应性。然而,由于治疗相关死亡率显著增加以及BMT特有的风险,如急性和慢性移植物抗宿主病以及严重的医源性慢性发病率,儿童骨髓移植的决策必须非常谨慎。如果考虑不匹配或无关供体移植,情况更是如此。本文讨论了根据当前BFM策略选择一种或另一种治疗方式的适应症。

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