Niu Jian-Hua, Yang Hua, Zhu Cheng-Ying, Zhang Qi, Sun Lu, Zhu Hai-Yan, Yao Zi-Long, Yu Li, Jing Yu
Department of Hematology and Oncology, The Forth People's Hospital of Jinan, Jinan 250031, Shandong Province, China; Department of Hematology, Chines PLA General Hospital, Beijing 100853, China.
Department of Hematology, DChines PLA General Hospital, Beijing 100853, China.
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2014 Jun;22(3):735-41. doi: 10.7534/j.issn.1009-2137.2014.03.030.
This study was aimed to investigate the pathology, MICM classification, PET/CT characteristics and therapeutical experience of subcutaneous soft tissue muscle gap lymphomatoid granulomatosis (LYG) through analysis of a cases of LYG. The pathologic changes of LYG were assayed by using immunohistochemistry method;the immuno-phenotypes were detected by flow cytometry. The nested multiplex PCR was used to detect the expression and mutation of abnormal genes; the real-time fluorescence quantitative PCR was used to detect the EBV-DNA copies. The clinical staging was performed by means of fluorodeoxyglucose positron emission tomography/computed tomography ((18)F-FDG PET/CT). The results showed that at onset of disease the clinical manifestations of patient presented only a mass in right thigh and swelling of right submandibular lymph nodes. However, PET/CT revealed that the abnormal image in multiple soft tissue accompanied by increasing metabolic activity (SUVmax = 12.8), these pathologic changes were involved in lung, thyroid, lymphonodes and stomach. The right thigh mass biopsy confirmed the histological diagnosis of grade II LYG. The bone marrow smear showed no abnormal tumor cell infiltration, the immunophenotyping detection revealed that the proportion of NK cells increased with phenotypic abnormality, the karyotype was 46, XY[24], the expression and mutation of abnormal gene not could be detected, and the EBV-DNA level was <10(2) copies/ml. After 2 cycles of treatment with rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone(R-CHOP), the images of increasing metabolic activity in subcutaneous soft tissue gap disappeared, but the partial increasing metabolism focus could be observed in soft tissue of left knee hollow. The patient achieved partial remission. It is concluded that LYG is an extremely rare hematopoietic malignancy, the incidence rate is very low. Subcutaneous soft tissue muscle gap LYG literature was not reported in domestic and foreign literatures.Its pathogenetic remains unclear. A standard treatment protocol for LYG has not yet been established. PET/CT can find more lesions that not could be found in the clinical examination. The (18)F-FDG PET/CT is an efficient tool for the LYG in diagnosis, staging and treatment. Therefore, increased SUV(max) in FDG-PET may be useful for diagnosis of LYG.
本研究旨在通过分析1例皮下软组织肌间隙淋巴瘤样肉芽肿病(LYG)的病例,探讨其病理学、MICM分类、PET/CT特征及治疗经验。采用免疫组织化学方法检测LYG的病理变化;应用流式细胞术检测免疫表型。采用巢式多重PCR检测异常基因的表达及突变情况;应用实时荧光定量PCR检测EBV-DNA拷贝数。通过氟脱氧葡萄糖正电子发射断层扫描/计算机断层扫描((18)F-FDG PET/CT)进行临床分期。结果显示,疾病初发时患者临床表现仅为右大腿肿块及右下颌下淋巴结肿大。然而,PET/CT显示多个软组织出现异常影像,伴有代谢活性增加(SUVmax = 12.8),这些病理改变累及肺、甲状腺、淋巴结及胃。右大腿肿块活检确诊为Ⅱ级LYG。骨髓涂片未见异常肿瘤细胞浸润,免疫表型检测显示NK细胞比例增加且表型异常,核型为46, XY[24],未检测到异常基因的表达及突变,EBV-DNA水平<10(2)拷贝/ml。经利妥昔单抗、环磷酰胺、阿霉素长春新碱和泼尼松(R-CHOP)方案治疗2个周期后,皮下软组织间隙代谢活性增加的影像消失,但左膝窝软组织可见部分代谢增高灶。患者达到部分缓解。结论认为,LYG是一种极其罕见的造血系统恶性肿瘤,发病率极低。国内外文献均未报道皮下软组织肌间隙LYG。其发病机制尚不清楚。尚未建立LYG的标准治疗方案。PET/CT能够发现临床检查中未发现的更多病灶。(18)F-FDG PET/CT是LYG诊断、分期及治疗的有效工具。因此,FDG-PET中SUV(max)升高可能有助于LYG的诊断。