皮下软组织肌肉间隙淋巴瘤样肉芽肿病1例PET-CT特点及临床表现病例分析  

Analysis of ^(18)F-FDG PET/CT Features and Clinical Manifestations in One Case of Subcutaneous Lymphomatoid Granulomatosis

作　　者：牛建花[1,2] 杨华[2] 朱成英[2] 张琪[2,3] 孙璐[4] 朱海燕[2] 姚子龙[2] 于力[2] 靖彧[2] 

机构地区：[1]山东省济南市第四人民医院血液肿瘤科,山东济南250031 [2]解放军总医院血液科,北京100853 [3]江苏省高淳县人民医院血液科,江苏高淳211300 [4]解放军总医院病理科,北京100853

出　　处：《中国实验血液学杂志》2014年第3期735-741,共7页Journal of Experimental Hematology

基　　金：解放军总医院临床科研扶持基金(2012FC-TSYS-2014)

摘　　要：本研究通过皮下软组织肌肉间隙淋巴瘤样肉芽肿病(lymphomatoid granulomatosis,LYG)的病例分析探讨LYG的病理、MICM(骨髓形态、免疫分型、分子生物学、细胞遗传学)、PET/CT和临床特点及治疗经验。应用病理免疫组织化学方法观察了LYG的病理改变,骨髓细胞涂片法观察骨髓细胞形态改变,流式细胞术检测免疫分型,多重巢式PCR检测异常基因表达和突变,实时荧光定量PCR(FQ-PCR)检测血清EBV-DNA含量,18氟-脱氧葡萄糖正电子发射断层显像/计算机断层成像(18F-FDG PET/CT)术明确临床分期。结果表明,患者发病时右大腿内侧有肿物和右颌下淋巴结肿大,但PET/CT检查发现患者全身多处软组织内有异常软组织影伴高代谢改变,且累及肺、甲状腺、淋巴结和胃;右大腿内侧肿物活检证实为淋巴瘤样肉芽肿病Ⅱ级,但骨髓细胞涂片显示无异常肿瘤细胞浸润;白血病免疫分型为NK细胞比例增高伴表型异常;染色体核型为46,XY[24];多重巢式PCR未检出异常基因表达和突变;FQ-PCR检测显示EBV-DNA<102copies/ml;2个周期R-CHOP方案(利妥昔单抗0.7 g d 0、环磷酰胺1.4 g d 1、表柔比星90 mg d 1、长春地辛4 mg d 1、泼尼松90 mg d 1-5)化疗后,PET-CT显示原双腿皮下软组织肌肉间隙高代谢结节样影基本消失,但左侧腘窝区域软组织内仍可见部分高代谢病灶,影像评估为PR。结论:淋巴瘤样肉芽肿病是一种罕见疾病,发病率极低;皮下软组织肌肉间隙淋巴瘤样肉芽肿病在国内外文献均未见报道;该病发病机制未明确,无标准治疗;PET/CT能发现临床体检不能发现的病变,能够再进行更准确的分期;PET/CT检查对淋巴瘤样肉芽肿病的辅助诊断、分期、疗效评价方面有重要价值。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 immunophenotypes were detected by flow cytometry. The nested multiplex PCR was used to detect the expression and mutation of abnormal genes; the real-time fluorescence guantitative PCR was used to detect the EBV-DNA copies. The clinical staging was performed by means of fluorodeoxyglucose positron emission tomography/computed tomography （18SF-FDG PET/CT）. The results showed that at onset of disearse the clinicai 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 inmultiple soft tissue accompanied by increasing metabolic activity （ SUVmax = 12.8 ）, these pathologic changes were involved in lung, thyroid, lymphonodes and stomach. The fight thigh mass biospy comfirmed 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 increated 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 〈 102 copies/ml. After 2 cycles of treatment with fituximab, cyclophosphamide, doxorubicin, vincristin and prednisolone （ R-CHOP）, the images of increasing metabolic activity in subcustaneous soft tissue gap dissappeared, but the partial increating 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 pathoge

关 键 词：皮下软组织 淋巴瘤样肉芽肿病 PET—CT特点 

分 类 号：R733.1[医药卫生—肿瘤]