Eosinophilia and bone lesion as clinical manifestations of aggressive systemic mastocytosis.
Eosinophilia and bone lesion as clinical manifestations of aggressive systemic mastocytosis.
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嗜酸性粒细胞增多和骨病变是侵袭性系统性肥大细胞增多症的临床表现。
DOI：
10.3960/jslrt.53.207
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发表时间：
2013
期刊：
影响因子：
--
通讯作者：
Takayuki Takahashi
中科院分区：
文献类型：
--
作者：
Emiko Sakane;T. Kodaka;Hiroko Tsunemine;K. Itoh;H. Akasaka;T. Kusama;K. Imaizumi;Masanori Taketomi;A. Sada;Y. Katayama;T. Itoh;Takayuki Takahashi
我们报告了一名患有侵袭性系统性肥大细胞增多症 (SM) 的患者，该患者表现出嗜酸性粒细胞增多和异常的破坏性骨病变。一名 43 岁女性，于 2011 年 2 月因椎体压缩性骨折、多发性溶骨性病变和嗜酸性粒细胞增多而转诊至我院。根据骨髓中肥大细胞异常、血清类胰蛋白酶水平高、以及包括椎体压缩性骨折在内的多发性溶骨性病变，诊断为侵袭性 SM。聚合酶链式反应及其产物测序鉴定c-kit突变的结果为阴性，甲磺酸伊马替尼未能改善患者的SM。随后她接受了干扰素-α 治疗，尽管严重的骨髓抑制导致无法继续使用足够剂量的该药物，但病情得到了显着改善。 2011年8月，患者突然出现下肢截瘫。磁共振成像显示 Th9 至 Th11 水平有硬膜外肿块病变，压迫脊髓。进行了紧急椎板切除术和随后的肿瘤放射治疗，但截瘫没有得到改善。根据术中获得的样本对硬膜外肿瘤进行组织病理学检查，证实了 SM 的诊断。进一步使用达沙替尼治疗，然后使用克拉屈滨治疗，但无明显改善，尽管后者在一定程度上减轻了嗜酸粒细胞增多；然而，她于2011年9月死于败血症。
We report a patient with aggressive systemic mastocytosis (SM), who exhibited eosinophilia and unusual destructive bone lesions. A 43-year-old female was referred to our hospital because of a vertebral compression fracture, multiple lytic bone lesions, and eosinophilia in February 2011. A diagnosis of aggressive SM was made on the basis of abnormal mast cells in the bone marrow, high serum tryptase levels, and multiple lytic bone lesions including vertebral compression fractures. Polymerase chain reaction and subsequent sequencing of its products to identify mutations of c-kit yielded negative results and imatinib mesylate failed to improve the SM of the patient. She was then treated with interferon-α, with considerable improvement of the disease, although severe myelosuppression prevented the continued administration of a sufficient dose of this agent. In August 2011, the patient suddenly developed paraplegia of the lower extremities. Magnetic resonance imaging demonstrated epidural mass lesions at the levels from Th9 to Th11, compressing the spinal cord. Emergent laminectomy and subsequent irradiation of the tumors were performed without improvement of the paraplegia. Histopathologic examination of the epidural tumors, from samples obtained intraoperatively, confirmed the diagnosis of SM. She was further treated with dasatinib and then cladribine without obvious improvement, although the latter reduced the eosinophilia to some extent ; however, she died of sepsis in September 2011.
影响因子：
158.5
作者：
Cools, J;DeAngelo, DJ;Gilliland, DG
通讯作者：
Gilliland, DG