Extramedullary involvement in patients with acute promyelocytic leukemia - A report of seven cases
Extramedullary involvement in patients with acute promyelocytic leukemia - A report of seven cases
复制标题
DOI：
10.1002/(sici)1097-0142(19981015)83:8
复制
发表时间：
1998-10-15
期刊：
影响因子：
6.2
通讯作者：
Biondi, A
中科院分区：
文献类型：
--
作者：
Liso, V;Specchia, G;Biondi, A
背景，在急性早幼粒细胞白血病（APL）患者中偶尔观察到髓外受累，但据说用全反式视黄酸（ATRA）治疗后比用细胞毒性药物治疗后更常见。文献中报道了 37 个有据可查的病例。方法。作者报告了 9 年间转诊至两个不同机构的 120 名成年 APL 患者中，通过细胞学、表型和分子分析记录的 7 名髓外 APL 患者。结果。在该 APL 系列中，120 例病例中有 7 例 (5.8%) 发生髓外疾病 (EMD)。 5 名患者的髓外部位是皮肤，1 名患者的髓外部位是皮肤，1 名患者的髓外部位是中枢神经系统，1 名患者的髓外部位是淋巴结。对四份皮肤样本和一份脑脊液样本进行了 PML/RAR α 重排的分子分析；所有患者的骨髓 (BM) 和 EMD 部位均表现出相同的分子模式。在 120 名患者中，61 名患者接受 ATRA 加化疗，59 名患者仅接受化疗。 38 名患者出现复发，其中 6 名患有 EMD； 1 名患者在 APL 发病时出现 EMD。在复发的 EMD 病例中，61 名患者中有 2 名接受了 ATRA 加化疗，59 名患者中有 4 名接受了单独化疗。结论。关于用 ATRA 治疗 APL 是否可能导致患者发生髓外复发存在一些争议。这项研究的数据没有证据表明接受 ATRA 治疗的 APL 患者可能更频繁地发生 EMD。癌症 1998；83：1522-8。 (C) 1998 年美国癌症协会。
BACKGROUND, Extramedullary involvement is only occasionally observed in patients with acute promyelocytic leukemia (APL) but has been said to occur more frequently after treatment with all-trans retinoic acid (ATRA) than after treatment with cytotoxic drugs. In the literature, 37 well-documented cases have been reported.METHODS. The authors report 7 patients with extramedullary APL documented by cytologic, phenotypic, and molecular analyses among 120 adult APL patients referred to two different institutions during a period of 9 years.RESULTS. In this APL series, extramedullary disease (EMD) occurred in 7 of 120 cases (5.8%). The extramedullary sites were the skin in five patients, the central nervous system in one, and the lymph nodes in one. Molecular analysis of the PML/RAR alpha rearrangement was performed on four samples of skin and one of CSF; all patients exhibited the same molecular pattern in the bone marrow (BM) and EMD sites. Of 120 patients, 61 were treated with ATRA plus chemotherapy and 59 with chemotherapy alone. Relapses were observed in 38 patients, 6 of whom had EMD; 1 patient had developed EMD at the onset of APL. Of the relapsed EMD cases, 2 of 61 patients had received ATRA plus chemotherapy and 4 of 59 had received chemotherapy alone.CONCLUSIONS. There is some controversy as to whether treatment of APL with ATRA may predispose patients to the development of extramedullary relapse. The data from this study do not contain evidence that EMD may occur more frequently in APL patients treated with ATRA. Cancer 1998;83:1522-8. (C) 1998 American Cancer Society.