Clinical characteristics of pediatric patients with myeloid sarcoma without bone marrow involvement in Japan
Clinical characteristics of pediatric patients with myeloid sarcoma without bone marrow involvement in Japan
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DOI：
10.1007/s12185-018-2492-5
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发表时间：
2018-10-01
影响因子：
2.1
通讯作者：
Ishida, Yasushi
中科院分区：
文献类型：
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作者：
Taga, Takashi;Imamura, Toshihiko;Ishida, Yasushi
骨髓肉瘤（MS）是一种罕见的肿瘤性疾病，通常与急性髓细胞白血病（AML）相关。儿童多发性硬化症很少受到关注，特别是在日本。我们对诊断为MS但无骨髓受累的日本儿童进行了全国范围的回顾性分析。入选标准为2000年1月1日至2013年12月31日期间年龄小于20岁的MS诊断。男性占优势（8：2），诊断MS的中位年龄为44岁。受累部位各不相同，包括皮肤（n=3）、头部和/或颈部（n=2）和多个部位（n=2）。对7名患者进行了染色体组型评估，其中1名患者携带t（8;21）和t（9;11）。4例患者在MS诊断后2- 55个月发生骨髓受累。所有患者均接受了原发AML化疗，2例患者在首次缓解时接受了HSCT。10例患者中有7例在首次化疗后无病生存50- 152个月（中位数93个月）。这项回顾性研究证实，在日本，无骨髓受累的儿童MS是一种非常罕见的疾病。MS患者对新发AML的治疗反应良好，首次缓解的HSCT并不适用于所有患者。
Myeloid sarcoma (MS) is a rare neoplastic condition that is often described in association with acute myeloid leukemia (AML). MS in childhood has received little attention, particularly in Japan. We carried out a nationwide retrospective analysis of Japanese children diagnosed with MS without bone marrow involvement. Inclusion criteria were diagnosis of MS at younger than 20years of age between January 1, 2000 and December 31, 2013. There was a predominance of males (8:2), and the median age at MS diagnosis was 4years. Sites of involvement varied and included skin (n=3), head and/or neck (n=2), and multiple sites (n=2). Karyotypes were evaluated in seven patients, with one individual carrying t(8;21) and t(9;11). Four patients developed bone marrow involvement 2-55months after diagnosis of MS. All patients received chemotherapy for de novo AML and two individuals received HSCT in first remission. Seven of ten patients survived for 50-152months (median, 93months) without disease after initial chemotherapy. This retrospective study confirmed that pediatric MS without bone marrow involvement in Japan is a very rare disease. MS patients responded favorably to therapies for de novo AML, and HSCT in first remission was not indicated for all patients.