Kai Tatsuyuki, Kimura Hideo, Shiga Yutaka, Ogawa Kazuei, Sato Hisashi, Maruyama Yukio
Hematology, Kita-Fukushima Medical Center, Fukushima, Japan.
Int J Hematol. 2006 May;83(4):337-40. doi: 10.1532/IJH97.05167.
Isolated extramedullary relapse is rare in patients with acute promyelocytic leukemia (APL) after allogeneic stem cell transplantation (SCT), and an optimal therapy for it has not been established. We describe a patient with APL who developed serially occurring extramedullary disease (EMD) after SCT. We confirmed that EMD had arisen from the recipient's APL blasts by detecting t(15;17) and PML/RARalpha from the tumor cell suspension. The patient displayed EMD 4 times at different sites. Administration of all-trans retinoic acid with local radiotherapy and with chemotherapy for the first to third EMDs resulted in regression of the tumors. However, these regimens did not prevent the subsequent occurrence of new EMD. For the fourth EMD, intravenous administration of arsenic trioxide followed by local radiotherapy resulted in the disappearance of EMD, and no further EMD has developed to date. In the present case, the bone marrow was in morphologic and molecular remission during the course of recurrent EMD. The accumulation of detailed cases is needed to elucidate the pathogenesis, predisposing factors, and optimal therapy for EMD in APL after SCT.
在接受异基因干细胞移植（SCT）的急性早幼粒细胞白血病（APL）患者中，孤立性髓外复发很少见，且尚未确立针对其的最佳治疗方法。我们描述了一名APL患者，该患者在SCT后出现了一系列髓外疾病（EMD）。通过从肿瘤细胞悬液中检测到t(15;17)和PML/RARα，我们证实EMD源自受者的APL原始细胞。该患者在不同部位出现了4次EMD。对于首次至第三次EMD，给予全反式维甲酸联合局部放疗及化疗后肿瘤消退。然而，这些方案未能阻止随后新的EMD发生。对于第四次EMD，静脉注射三氧化二砷后联合局部放疗导致EMD消失，且迄今为止未再出现新的EMD。在本病例中，在复发性EMD过程中骨髓处于形态学和分子学缓解状态。需要积累更多详细病例以阐明SCT后APL中EMD的发病机制、易感因素及最佳治疗方法。
