관해 유도 후 글리벡과 동종 조혈모세포 이식으로 치료한 필라델피아 염색체 양성 급성 골수성백혈병 3예
Cases of Ph+ AML treated with Glivec & allogeneic HSCT
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필라델피아 염색체 양성 일차성 급성 골수성백혈병은 보편적으로 치료에 반응을 잘 하지 않아 예후가 나쁘지만, 글리벡을 항암화학요법과 병용할 경우, 관해를 유도하고 유지하는데에 도움이 될 것이라고 소개되었다. 장기적인 관해 유지를 위해서는 궁극적으로 동종 조혈모세포 이식을 고려해야 하나, 국내외적으로 증례가 드물고 예후가 나쁘기 때문에 필라델피아 양성 일차성 급성 골수성백혈병에 대한 표준적인 치료법을 제시할만한 근거가 부족하다. 글리벡을 병용하는 것에 대한 몇몇 국외 증례가 있으나, 근거 확립을 위해 더 많은 증례 보고가 필요하리라 생각되며, 저자들이 제시한 것처럼 항암 화학요법과 글리벡의 중간 요법으로 안정된 관해 상태를 유지하고 동종 조혈모세포 이식을 시행하여 효과적인 치료 결과를 기대할 수 있을 것이다.
Abstract
The Ph-chromosome is the hallmark of chronic myelogenous leukemia (CML), but it has also been reported infrequently for de novo AML. This cytogenetic variant of AML is characterized by a poor prognosis associated with extraordinary drug resistance. Experience with the use of imatinib mesylate for Ph-chromosome-positive de novo AML is limited because of the rarity of the disorder. Thus, allogeneic hematopoietic stem cell transplantation (HSCT), as a post-remission therapy, should be considered. In three patients with Ph-chromosome-positive AML, we achieved complete remission with induction chemotherapy and the use of an imatinib cycle as an interim therapy. Subsequently, all patients received consolidation chemotherapy followed by imatinib cycles, bridging the time to allogeneic HSCT. All patients were successfully transplanted, and have been in excellent condition, with a median follow-up duration of 15 months (range, 2-25 months). We report on these cases and present the treatment strategy of induction chemotherapy, maintenance with imatinib mesylate, and final allogeneic HSCT as a representative paradigm for the treatment of Ph-chromosome-positive de novo AML.
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