肺原发性NK/T细胞淋巴瘤临床病理观察
|
摘要
目的探讨肺原发性NK/T细胞淋巴瘤(PPNKTCL)的临床病理特点及鉴别诊断,提高对PPNKTCL的认识。方法观察1例PPNKTCL的临床病理特征和免疫组化表型,原位杂交检测EB病毒,并复习国内外文献。结果患者女性,27岁。以发热、咳嗽、呼吸困难为主要临床表现,抗生素治疗无效。影像学示双侧肺片团状实变影伴左则胸腔积液。组织学,肺支气管和实质内异型淋巴细胞,以血管中心性浸润生长为主,伴血管破坏和凝固性坏死。免疫组化:肿瘤细胞CD3ε、CD56、粒酶B(+),CD3、CD79a、CD20、CgA、NSE、EMA、AE1/AE3、HMB45(-)。EBER1/2原位杂交(+)。患者发病后2个月死亡。结论 PPNKTCL是罕见的结外侵袭性淋巴瘤,预后极差。临床上出现难以控制的肺炎而无明确感染源,伴肺部阴影和胸腔积液时应想到本病。
Objective To investigate the clinicopathological characteristics and the differential diagnosis of the primary pulmonary NK / T-cell lymphoma( PPNKTCL),and to improve our understanding of PPNKTCL. Methods The clinical and pathological characteristics and the immunohistochemical phenotype of one case of PPNKTCL were analyzed,and then the EB virus was detected by in situ hybridization. Finally,comprehensive discussion was performed. Results A 27-year- old female patient presented with fever,cough,dyspnea and ineffective to antibiotics. CT imaging showed missionshaped opacities in bilateral lung with left pleural effusion. Histological observation showed that the atypical lymphocytes in bronchi and parenchyma of the lung were invasively distributed in blood vessels and associated with vascular injury and coagulative necrosis. Immunohistochemical results showed that CD3ε,CD56 and granzyme B were positive in this patient, while CD3,CD79a,CD20,CgA,NSE,EMA,AE1 / AE3 and HMB45 were negative. Positive EBER1 / 2 was detected by in situ hybridization. The patient died the second month after the diagnosis. Conclusion PPNKTCL is a rare extranodal aggressive lymphoma with poor prognosis. Hard-to-control pneumonia,without a clear source of infection,associated with pulmonary shadows and pleural effusion may be a critical clinical diagnostic clue of PPNKTCL.
Objective To investigate the clinicopathological characteristics and the differential diagnosis of the primary pulmonary NK / T-cell lymphoma( PPNKTCL),and to improve our understanding of PPNKTCL. Methods The clinical and pathological characteristics and the immunohistochemical phenotype of one case of PPNKTCL were analyzed,and then the EB virus was detected by in situ hybridization. Finally,comprehensive discussion was performed. Results A 27-year- old female patient presented with fever,cough,dyspnea and ineffective to antibiotics. CT imaging showed missionshaped opacities in bilateral lung with left pleural effusion. Histological observation showed that the atypical lymphocytes in bronchi and parenchyma of the lung were invasively distributed in blood vessels and associated with vascular injury and coagulative necrosis. Immunohistochemical results showed that CD3ε,CD56 and granzyme B were positive in this patient, while CD3,CD79a,CD20,CgA,NSE,EMA,AE1 / AE3 and HMB45 were negative. Positive EBER1 / 2 was detected by in situ hybridization. The patient died the second month after the diagnosis. Conclusion PPNKTCL is a rare extranodal aggressive lymphoma with poor prognosis. Hard-to-control pneumonia,without a clear source of infection,associated with pulmonary shadows and pleural effusion may be a critical clinical diagnostic clue of PPNKTCL.
引文
[1]Jaffe ES,Harris NL,Stein H.World Health Organization classification of tumors.Pathology and genetics of tumors of haematopoietic and lymphoid tissues[M].Lyon:IARC Press,2001.204-207.
[2]Sun J,Yang Q,Lu Z.Distribution of lymphoid neoplasms in china:analysis of 4638 cases according to the World Health Organization classification[J].Am J Clin Pathol,2012,138(3):429-434.
[3]Brian W,Davis MD,Beasley MB,et al.Primary pulmonary natural killer/T-cell lymphoma presenting as nonresolving pneumonia[J].Chest,2010,138(4):18A.
[4]曹孟淑,蔡后荣,印洪林,等.原发性肺自然杀伤/T细胞淋巴瘤2例临床分析[J].中华结核和呼吸杂志,2008,31(2):120-124.
[5]Laohaburanakit P,Hardin KA.NK/T-cell lymphoma of the lung:a case report and review of literature[J].Thoras,2006,61(3):267-270.
[6]Lee BH,Kim SY,Kim MY,et al.CT of nasal-type T/NK cell lymphoma in the lung[J].J Thorac Imaging,2006,21(1):37-39.
[7]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.
[8]Cadranel J,Wislez M,Antoine M.Primary pulmonary lymphoma[J].Eur Respir J,2002,20(3):750-762.
[9]Koss MN,Hochnoblzer L,Nichols PW,et al.Primary nonHodgkin’s lymphoma and pseudolymphoma of lung:a study of161 patients[J].Hum Pathol,1983,14(13):1024-1038.
[10]Cordier JF,Chailleux E,Lauque D.Primary pulmonary lymphomas:a clinical study of 70 cases in nonimmuno compromised patients[J].Chest,1993,103(1):201-208.
[11]李丹,李甘地,刘卫平,等.原发性肺恶性淋巴瘤15例临床病理分析[J].山西医科大学学报,2004,35(6):589-592.
[12]孙璐,石怀银,陈薇,等.原发性肺外周T细胞淋巴瘤临床病理观察[J].诊断病理学杂志,2012,19(2):135-139.
[13]Lee KS,Kim Y,Primack SL.Imaging of pulmonary lymphoma[J].AJR AM J Roentgenol,1997,168(2):339-345.
[2]Sun J,Yang Q,Lu Z.Distribution of lymphoid neoplasms in china:analysis of 4638 cases according to the World Health Organization classification[J].Am J Clin Pathol,2012,138(3):429-434.
[3]Brian W,Davis MD,Beasley MB,et al.Primary pulmonary natural killer/T-cell lymphoma presenting as nonresolving pneumonia[J].Chest,2010,138(4):18A.
[4]曹孟淑,蔡后荣,印洪林,等.原发性肺自然杀伤/T细胞淋巴瘤2例临床分析[J].中华结核和呼吸杂志,2008,31(2):120-124.
[5]Laohaburanakit P,Hardin KA.NK/T-cell lymphoma of the lung:a case report and review of literature[J].Thoras,2006,61(3):267-270.
[6]Lee BH,Kim SY,Kim MY,et al.CT of nasal-type T/NK cell lymphoma in the lung[J].J Thorac Imaging,2006,21(1):37-39.
[7]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.
[8]Cadranel J,Wislez M,Antoine M.Primary pulmonary lymphoma[J].Eur Respir J,2002,20(3):750-762.
[9]Koss MN,Hochnoblzer L,Nichols PW,et al.Primary nonHodgkin’s lymphoma and pseudolymphoma of lung:a study of161 patients[J].Hum Pathol,1983,14(13):1024-1038.
[10]Cordier JF,Chailleux E,Lauque D.Primary pulmonary lymphomas:a clinical study of 70 cases in nonimmuno compromised patients[J].Chest,1993,103(1):201-208.
[11]李丹,李甘地,刘卫平,等.原发性肺恶性淋巴瘤15例临床病理分析[J].山西医科大学学报,2004,35(6):589-592.
[12]孙璐,石怀银,陈薇,等.原发性肺外周T细胞淋巴瘤临床病理观察[J].诊断病理学杂志,2012,19(2):135-139.
[13]Lee KS,Kim Y,Primack SL.Imaging of pulmonary lymphoma[J].AJR AM J Roentgenol,1997,168(2):339-345.