肺部NK/T细胞淋巴瘤3例临床病理分析
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摘要
目的探讨肺部NK/T细胞淋巴瘤(NK/T-cell lymphoma,ENKTL)的临床病理学特征、诊断及鉴别诊断。方法回顾性分析3例肺部ENKTL的临床病理学和免疫表型特征、原位杂交结果及鉴别诊断。结果 3例中2例女性,1例男性,发病年龄52~68岁,临床表现及影像学无特异性。镜下见肿瘤侵犯肺实质及支气管,以血管为中心浸润和血管破坏性生长为特征,伴不同程度的炎症反应及凝固性坏死。免疫表型:肿瘤细胞均表达CD3、CD56、细胞毒性标志物(TIA-1、Perforin、Gran-B),2例表达CD5、CD30。3例EBER原位杂交检测均阳性。2例行TCR基因重排,结果均阴性。结论肺部ENKTL临床罕见,诊断难度大,预后差,明确诊断需综合病理组织学、免疫表型及分子病理学检测。
Purpose To improve the knowledge of the clinicopathologic features of pulmonary extranodal NK/T-cell lymphoma( ENKTL). Methods Three cases of pulmonary ENKTL were analyzed retrospectively,medical recordings,imaging manifestations,pathological features,immunophenotypes,EBER in situ hybridizations and other related literatures were reviewed.Results The group was composed of 1 man and 2 women with ages ranging from 52 to 68 years. The clinical symptoms and computed tomography( CT) manifestations were nonspecific.Histological observation showed that atypical lymphocytes in bronchi and parenchyma of the lung were invasively distributed.The neoplasms presented angiocentric and angiodestructivegrowth patterns with different degrees of inflammatory response and necrosis. For the immunophenotypes,all cases were positive for CD3,CD56 and cytotoxic granule( TIA-1,Perforin,GranB). The positive ratios of CD5 and CD30 were both 2/3. All 3 cases showed positive in situ hybridization for Epstein-Barr virusencoded small RNA( EBER). Conclusion Pulmonary extranodal NK/T-cell lymphoma is very rare with poor prognosis and difficult to diagnose. The immunohistochemical staining and molecular pathological technology may be helpful for the diagnosis and differential diagnosis of the tumors.
Purpose To improve the knowledge of the clinicopathologic features of pulmonary extranodal NK/T-cell lymphoma( ENKTL). Methods Three cases of pulmonary ENKTL were analyzed retrospectively,medical recordings,imaging manifestations,pathological features,immunophenotypes,EBER in situ hybridizations and other related literatures were reviewed.Results The group was composed of 1 man and 2 women with ages ranging from 52 to 68 years. The clinical symptoms and computed tomography( CT) manifestations were nonspecific.Histological observation showed that atypical lymphocytes in bronchi and parenchyma of the lung were invasively distributed.The neoplasms presented angiocentric and angiodestructivegrowth patterns with different degrees of inflammatory response and necrosis. For the immunophenotypes,all cases were positive for CD3,CD56 and cytotoxic granule( TIA-1,Perforin,GranB). The positive ratios of CD5 and CD30 were both 2/3. All 3 cases showed positive in situ hybridization for Epstein-Barr virusencoded small RNA( EBER). Conclusion Pulmonary extranodal NK/T-cell lymphoma is very rare with poor prognosis and difficult to diagnose. The immunohistochemical staining and molecular pathological technology may be helpful for the diagnosis and differential diagnosis of the tumors.
引文
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[13]Au W Y,Weisenburger D D,Intragumtornchai T,et al.Clinical differences between nasal and extranasal natural killer/T-cell lym-phoma:a study of 136 cases from the international peripheral T-cell lymphoma project[J].Blood,2009,113(17):3931-3937.
[2]Ding W,Wang J,Zhao S,et al.Clinicopathological study of pulmonary extranodal nature killer/T-cell lymphoma,nasal type and literature review[J].Pathol Res Pract,2015,211(7):544-549.
[3]Chilosi M,Zinzani P L,Poletti V.Lymphoproliferative lung disorders[J].Semin Respir Crit Care Med,2005,26(5):490-501.
[4]Lee B H,Kim S Y,Kim M Y,et al.CT of nasal-type T/NK cell lymphoma in the lung[J].J Thorac Imaging,2006,21(1):37-39.
[5]Gong L.Identification of genuine primary pulmonary NK cell lymphoma via clinicopathologic observation and clonality assay[J].Diagn Pathol,2013,8(1):1-7.
[6]Laohaburanakit P.NK/T cell lymphoma of the lung:a case report and review of literature[J].Thorax,2006,61(3):267-270.
[7]Gui W,Yang B,Shen Q,et al.Successful treatment with L-asparaginase-based regimen for primary pulmonary NK/T cell lymphoma:a case report and review of the literature[J].Clin Respr J,2015,9(4):493-496.
[8]苏欣,宋勇,施毅,等.原发性肺结外鼻型NK/T细胞淋巴瘤一例临床分析[J].临床内科杂志,2006,23(2):106-109.
[9]肖永龙,张德平,王益华.以肺为首发部位的NK/T细胞淋巴瘤二例并文献复习[J].中华内科杂志,2007,46(12):988-991.
[10]徐德,林博宁,付劲锋.肺原发性NK/T细胞淋巴瘤临床病理观察[J].诊断病理学杂志,2014,21(4):238-241.
[11]Koss M N,Hochholzer L,Nichols P W,et al.Primary nonHodgkin's lymphoma and pseudolymphoma of lung:a study of 161patients[J].Hum Pathol,1983,14(12):1024-1038.
[12]许文静,刘伟,郑智勇.以子宫颈为首发病灶的结外鼻型NK/T细胞淋巴瘤1例[J].临床与实验病理学杂志,2015,31(2):239-240.
[13]Au W Y,Weisenburger D D,Intragumtornchai T,et al.Clinical differences between nasal and extranasal natural killer/T-cell lym-phoma:a study of 136 cases from the international peripheral T-cell lymphoma project[J].Blood,2009,113(17):3931-3937.