Spontaneous remission of 2-chlorodeoxyadenosine (2-CdA)-related secondary myelodysplastic syndrome in a patient with refractory Langerhans cell histiocytosis
Spontaneous remission of 2-chlorodeoxyadenosine (2-CdA)-related secondary myelodysplastic syndrome in a patient with refractory Langerhans cell histiocytosis
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难治性朗格汉斯细胞组织细胞增多症患者 2-氯脱氧腺苷 (2-CdA) 相关继发性骨髓增生异常综合征的自发缓解
DOI：
10.1007/s12185-013-1343-7
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发表时间：
2013
影响因子：
2.1
通讯作者：
K. Kobayashi
中科院分区：
文献类型：
--
作者：
D. Suzuki;R. Kobayashi;Hirozumi Sano;K. Kishimoto;K. Yasuda;M. Nakanishi;T. Nagashima;K. Kobayashi
朗格汉斯细胞组织细胞增生症（LCH）有时对常规化疗有抵抗力，2-氯脱氧腺苷（2-CdA）治疗作为难治性或复发性LCH的挽救治疗越来越重要。继发性恶性肿瘤，如急性髓性白血病和骨髓增生异常综合征（MDS）由于2-CdA最近有报道。然而，迄今为止还没有2-CdA相关的继发性MDS自发缓解的病例报告。在这里，我们报告的情况下，一个1岁的男孩与枕部肿瘤谁被诊断为LCH活检和接受化疗。然而，这种疾病复发，并成为化疗难治性。他接受了2-CdA治疗，有效。然而，在治疗开始后6个月，他发生了MDS，染色体异常为7 q −。经过1年的观察，没有任何干预，他的血液学检查结果恢复正常，染色体异常也消失了。据我们所知，这是第一次报告的2-CdA相关的MDS自发血液学缓解。
Langerhans cell histiocytosis (LCH) is sometimes resistant to conventional chemotherapies, and treatment with 2-chlorodeoxyadenosine (2-CdA) is gaining importance as a salvage treatment for refractory or recurrent LCH. Secondary malignancies such as acute myelogenous leukemia and myelodysplastic syndrome (MDS) due to 2-CdA have recently been reported. However, there have been no reports to date of cases of 2-CdA-related secondary MDS in which spontaneous remission was achieved. Here, we report the case of a 1-year-old boy with an occipital tumor who was diagnosed with LCH by biopsy and underwent chemotherapy. However, the disease relapsed and became refractory to chemotherapy. He received 2-CdA treatment, which was effective. However 6 months after the start of treatment, he developed MDS with chromosomal abnormality of 7q−. After 1-year observation without any intervention, his hematological findings had returned to normal, and the chromosomal abnormality had also disappeared. To our knowledge, this is the first report of 2-CdA-related MDS with spontaneous hematological remission.