Extramedullary acute promyelocytic leukemia
Extramedullary acute promyelocytic leukemia
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髓外急性早幼粒细胞白血病
DOI：
10.1002/(sici)1097-0142(19970701)79:11
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发表时间：
1997
期刊：
影响因子：
6.2
通讯作者：
F. Oberling
中科院分区：
文献类型：
--
作者：
F. Maloisel;J. Kurtz;F. Oberling
我们饶有兴趣地阅读了Wiernik等人关于急性早幼粒细胞白血病（APL）患者髓外白血病浸润的病例报告和综述。本文报告了2例新病例，并对24例文献资料进行了分析。髓外浸润主要部位为皮肤15例，中枢神经系统5例。然而，在白血病的自然史中，虽然这些定位和睾丸受累是最常见的髓外定位，但胃肠道部位是例外。我们发表了一例在一线化疗后7个月首次复发的患者结肠定位。简而言之，一名42岁白人男性在接受左柔比星和阿糖胞苷治疗后7个月的APL完全缓解（CR）后入院。在报告时，他的骨髓研究是APL的诊断。他的初始白细胞计数为4000/mL，白细胞计数差异未显示任何母细胞或高颗粒早幼粒细胞。在初始治疗前和复发时，他的骨髓核型为46XY， t(15,17)， ú80%白血病细胞为CD13，但未检测CD56。复发患者给予左柔比星和全反式维甲酸（ATRA） 45 mg/m /d治疗。第5天，患者出现粒细胞减少，腹痛和明显腹胀，粪便和放屁完全停止。腹膜显示大肠大量气体膨胀，未见液体，提示急性结肠假性梗阻。结肠镜减压显示沿乙状结肠、左结肠和横结肠弥漫性溃疡病变，最大尺寸约5毫米，而活检样本的组织学检查证实粘膜和粘膜下层有白血病浸润。在免疫组织学检查中，血液、尿液和活组织检查均未见巨细胞病毒。继续ATRA治疗，第二次CR达到完全解决消化症状；1个月后，对照纤维结肠镜检查显示溃疡完全消退。然而，患者11个月后复发并死于脑膜出血。我们目前的病例和Wiernik等人报道的病例需要一些评论。ATRA的使用代表了APL患者治疗的重大突破，并增加了无病生存期和总生存期。然而，据我们所知，迄今为止没有相关数据证明ATRA治疗使患者易发生髓外复发。对11例细胞遗传学记录的APL患者的简单分析显示，ATRA诱导后5例，化疗后5例，两种治疗后1例。Wiernik等报道的2例病例均表现为白细胞增多，这是导致复发和髓外复发的重要因素。此外，extra-的优势
We read with interest the case report and review by Wiernik et al. regarding extramedullary leukemic infiltration in patients with acute promyelocytic leukemia (APL). In this article, 2 new cases were reported and 24 well documented instances were also analyzed. The main sites of extramedullary infiltration were the skin in 15 cases and the central nervous system in 5. However, although these localizations and testicular involvement are the most frequent extramedullary localizations during the natural history of leukemia, gastrointestinal sites are exceptional. We have published a case of colonic localization in a patient with a first recurrence 7 months after front-line chemotherapy. Briefly, a 42-year-old white male was admitted with recurrent APL after a 7-month complete remission (CR) induced with zorubicine and cytarabine in accordance with the APL91 regimen. At presentation, his bone marrow studies were diagnostic of APL. His initial leukocyte count was 4000/mL and a differential leukocyte count did not reveal any blasts or hypergranulated promyelocytes. Prior to initial therapy and at the time of recurrence his bone marrow karyotype was 46XY, t(15,17) and ú80% leukemic cells were CD13, but CD56 was not tested. His recurrence was treated with zorubicine and all-trans retinoic acid (ATRA), 45 mg/m /day. On Day 5, the patient had granulopenia and developed abdominal pain and marked distension with complete arrest of feces and flatus. The abdominal film showed a massive gaseous distension of the large bowel, without any fluid content, suggestive of acute colonic pseudoobstruction. Colonoscopic decompression was performed and revealed diffuse ulcerated lesions of approximately 5-mm in greatest dimension along the sigmoid, left, and transverse colon, whereas histologic examination of biopsy samples confirmed leukemic infiltration of the mucosa and submucosa. Cytomegalovirus was absent from blood, urine, and biopsies at immunohistologic assays. Treatment with ATRA was continued and a second CR was achieved with complete resolution of digestive symptoms; after 1 month, a control fiber colonoscopy showed a total regression of ulcerations. Nevertheless, the patient recurred 11 months later and died of meningeal hemorrhage. Our present case and those reported by Wiernik et al. require some comments. The use of ATRA represents a major breakthrough in the treatment of patients with APL and increases the disease free and overall survival. However, to our knowledge, to date there are no relevant data to prove that ATRA therapy predisposes patients to the development of extramedullary recurrence. The simple analysis of the 11 patients with cytogenetically documented APL shows 5 cases after ATRA induction, 5 after chemotherapy, and 1 after both therapies. The two cases reported by Wiernik et al. both presented with hyperleukocytosis, which is an important factor for recurrence and extramedullary recurrence. Moreover, the predominance of extra-