A case report of pulmonary lymphomatoid granulomatosis confirmed by autopsy and review of the literature_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

 ZHANG Youyi ¹ , LI Juan ²

1. Department of Radiology, Sichuan Academy of Medical Sciences, Sichuan Province People＇s Hospital, Chengdu, Sichuan 610072, P. R. China;
2. Department of Pathology, Sichuan Academy of Medical Sciences, Sichuan Province People＇s Hospital, Chengdu, Sichuan 610072, P. R. China;

Corresponding?author：

ZHANG Youyi, Email: zhangyouyi108@163.com

Keywords：

Lung; Lymphomatoid granulomatosis; Diagnosis; Prognosis

DOI：

10.7507/1671-6205.201811036

Video：

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Objective To investigate the clinical, imaging features, diagnosis, treatment and prognosis of pulmonary lymphomatoid granulomatosis (PLG).Methods A case of PLG confirmed by autopsy and pathology was reported, and the related literature was reviewed.Results A 44-year-old male patient presented with cough, expectoration and shortness of breath as the main symptoms. The imaging findings of lung CT were diffuse vascular nodular and patchy changes in the lungs, especially in the lower lung. Clinical symptoms were not relieved with anti-infection and symptomatic treatment. The patient's condition gradually exacerbated and finally died. The autopsy revealed PLG with pathological grade Ⅲ. The infiltration of lymphocytes centered with blood vessel and necrosis were observed under light microscope. EBER in situ hybridization was positive. Totally 28 Chinese articles and 34 foreign articles were screened out. Literature review suggested that PLG usually occurred in middle-aged men and was associated with EBV infection. It is frequent with immunodepression. Its clinical symptoms were atypical. The main imaging manifestations were multiple nodules and masses more frequent along the bronchovascular bundle. A diagnosis of PLG depended on pathological histology, immunohistochemistry and EBER in situ hybridization. Surgery was the main treatment for local lesions. The patients of grade I and Ⅱ with rapid progress and all grade Ⅲ were usually treated with RCHOP regimen combined with chemotherapy. The prognosis was correlated with grading. Grade Ⅲ was aggressive and the prognosis was poor.Conclusions PLG is an atypical pulmonary lymphoproliferative disorder. It can be seen in many immunosuppressive diseases. The CT imaging features of PLG have certain characteristics, but the final diagnosis should be combined with pathological diagnosis. Some patients can be relieved by treatment, and patients with grade Ⅲ have poor prognosis.

Citation： ZHANG Youyi, LI Juan. A case report of pulmonary lymphomatoid granulomatosis confirmed by autopsy and review of the literature. Chinese Journal of Respiratory and Critical Care Medicine, 2019, 18(6): 567-572. doi: 10.7507/1671-6205.201811036 Copy

1.	Liebow AA, Carrington CR, Friedman PJ. Lymphomatoid granulomatosis. Hum Pathol, 1972, 3(4): 457-558.
2.	Sykorová A, Campr V, Ka?parová P, et al. Lymphomatoid granulomatosis - the past and present. Vnitr Lek, 2014, 60(3): 225-238.
3.	Swerdlow SH, Campo E, Harris NL, et al. World Health Organization classification of tumor haematopoietic and lymphoid tissue. 4th ed. Lyon: IARC Press, 2017, 312-314.
4.	Haque AK, Myers JL, Hudnall SD, et al. Pulmonary lymphomatoid granulomatosis in acquired immunodeficiency syndrome: lesions with Epstein-Barr virus infection. Mod Pathol, 1998, 11(4): 347-356.
5.	Martínez-Deltoro A, Herrera Lara S, Mogrovejo Calle S. Pulmonary manifestation of lymphomatoid granulomatosis. Arch Bronconeumol, 2015, 51(11): 605-606.
6.	秦軍, 高媛, 杜志強, 等. 肺淋巴瘤樣肉芽腫病一例. 中國呼吸與危重監護雜志, 2006, 5(5): 388-389.
7.	王承志, 章明放. 肺淋巴瘤樣肉芽腫病研究進展. 醫學綜述, 2011, 17(23): 3569-3571.
8.	李龍蕓, 劉彤華. 肺淋巴瘤樣肉芽腫病二例報告及文獻復習. 中華結核和呼吸雜志, 1991, 14(3): 162-164.
9.	孫紅, 白友賢, 蔡祖龍. 肺淋巴瘤樣肉芽腫的 X 線與 CT 診斷. 中華放射學雜志, 1998, 32(3): 212.
10.	馮瑞娥, 劉鴻瑞, 劉彤華, 等. 肺淋巴瘤樣肉芽腫病的免疫表型及基因重排研究. 中華病理學雜志, 2011, 40(7): 460-464.
11.	龐濤, 王新怡, 柳澄, 等. 肺淋巴瘤樣肉芽腫的 CT 特征. 醫學影像學雜志, 2010, 20(9): 1300-1302.
12.	Guinee D, Jaffe E, Kingma D, et al. Pulmonary lymphomatoid granulomatosis. Evidence for a proliferation of Epstein-Barr virus infected B-lymphocytes with a prominent T cell component and vasculitis. Am J Surg Pathol, 1994, 18(8): 753-764.
13.	Medeiros LJ, Jaffe ES, Chen YY, et al. Localization of Epstein-Barr viral genomes in angiocentric immunoproliferative lesions. Am J Surg Pathol, 1992, 16(5): 439-447.
14.	黃建偉. 肺淋巴瘤樣肉芽腫 3 例臨床病理分析. 現代醫藥衛生, 2018, 34(5): 799-800.
15.	楊春蓉, 鄭曉丹, 胡余昌, 等. 肺淋巴瘤樣肉芽腫 5 例臨床病理分析. 臨床與實驗病理學雜志, 2016, 32(8): 924-930.
16.	邱鵬. 肺淋巴瘤樣肉芽腫的臨床病理與分析兩例報告. 實用心腦肺血管病雜志, 2012, 20(6): 1089-1091.
17.	Wilson WH, Kingma DW, Raffeld M, et al. Association of lymphomatoid granulomatosis with Epstein-Barr viral infection of B lymphocytes and response to interferon-alpha 2b. Blood, 1996, 87(11): 4531-4537.
18.	Jung KH, Sung HJ, Lee JH, et al. A case of pulmonary lymphomatoid granulomatosis successfully treated by combination chemotherapy with rituximab. Chemotherapy, 2009, 55(5): 386-390.
19.	Fauci AS, Haynes BF, Costa J, et al. Lymphomatoid granulomatosis. Prospective clinical and therapeutic experience over 10 years. N Engl J Med, 1982, 306(2): 68-74.
20.	Katzenstein AL, Carrington CB, Liebow AA. Lymphomatoid granulomatosis: a clinicopathologic study of 152 cases. Cancer, 1979, 43(1): 360.
21.	Ankita G, Shashi D. Pulmonary lymphomatoid granulomatosis - a case report with review of literature. Indian J Surg Oncol, 2016, 7(4): 484-487.
22.	Gon?alves A, Wen X, Campainha S, et al. Lymphomatoid granulomatosis - a rare pulmonary lymphoproliferative disease. Rev Port Pneumol, 2016, 22(4): 248-249.
23.	Jonathan H. Chung, Jo-Anne O. Shepard, et al Lymphomatoid granulomatosis: CT and FDG-PET findings. Korean J Radiol, 2011, 12(6): 671-678.
24.	繆偉, 朱華淵, 徐衛. 世界衛生組織 2016 年淋巴瘤分類更新解讀. 中國實用內科雜志, 2016, 36(8): 647-653.
25.	Song JY, Pittaluga S, Dunleavy K, et al. Lymphomatoid granulomatosis-a single institute experience: pathologic findings and clinical correlations. Am J Surg Pathol, 2015, 39(2): 141-156.
26.	Borie R, Wislez M, Antoine M, et al. Lymphoproliferative disorders of the lung. Respiration, 2017, 94(2): 157-175.

1. Liebow AA, Carrington CR, Friedman PJ. Lymphomatoid granulomatosis. Hum Pathol, 1972, 3(4): 457-558.
2. Sykorová A, Campr V, Ka?parová P, et al. Lymphomatoid granulomatosis - the past and present. Vnitr Lek, 2014, 60(3): 225-238.
3. Swerdlow SH, Campo E, Harris NL, et al. World Health Organization classification of tumor haematopoietic and lymphoid tissue. 4th ed. Lyon: IARC Press, 2017, 312-314.
4. Haque AK, Myers JL, Hudnall SD, et al. Pulmonary lymphomatoid granulomatosis in acquired immunodeficiency syndrome: lesions with Epstein-Barr virus infection. Mod Pathol, 1998, 11(4): 347-356.
5. Martínez-Deltoro A, Herrera Lara S, Mogrovejo Calle S. Pulmonary manifestation of lymphomatoid granulomatosis. Arch Bronconeumol, 2015, 51(11): 605-606.
6. 秦軍, 高媛, 杜志強, 等. 肺淋巴瘤樣肉芽腫病一例. 中國呼吸與危重監護雜志, 2006, 5(5): 388-389.
7. 王承志, 章明放. 肺淋巴瘤樣肉芽腫病研究進展. 醫學綜述, 2011, 17(23): 3569-3571.
8. 李龍蕓, 劉彤華. 肺淋巴瘤樣肉芽腫病二例報告及文獻復習. 中華結核和呼吸雜志, 1991, 14(3): 162-164.
9. 孫紅, 白友賢, 蔡祖龍. 肺淋巴瘤樣肉芽腫的 X 線與 CT 診斷. 中華放射學雜志, 1998, 32(3): 212.
10. 馮瑞娥, 劉鴻瑞, 劉彤華, 等. 肺淋巴瘤樣肉芽腫病的免疫表型及基因重排研究. 中華病理學雜志, 2011, 40(7): 460-464.
11. 龐濤, 王新怡, 柳澄, 等. 肺淋巴瘤樣肉芽腫的 CT 特征. 醫學影像學雜志, 2010, 20(9): 1300-1302.
12. Guinee D, Jaffe E, Kingma D, et al. Pulmonary lymphomatoid granulomatosis. Evidence for a proliferation of Epstein-Barr virus infected B-lymphocytes with a prominent T cell component and vasculitis. Am J Surg Pathol, 1994, 18(8): 753-764.
13. Medeiros LJ, Jaffe ES, Chen YY, et al. Localization of Epstein-Barr viral genomes in angiocentric immunoproliferative lesions. Am J Surg Pathol, 1992, 16(5): 439-447.
14. 黃建偉. 肺淋巴瘤樣肉芽腫 3 例臨床病理分析. 現代醫藥衛生, 2018, 34(5): 799-800.
15. 楊春蓉, 鄭曉丹, 胡余昌, 等. 肺淋巴瘤樣肉芽腫 5 例臨床病理分析. 臨床與實驗病理學雜志, 2016, 32(8): 924-930.
16. 邱鵬. 肺淋巴瘤樣肉芽腫的臨床病理與分析兩例報告. 實用心腦肺血管病雜志, 2012, 20(6): 1089-1091.
17. Wilson WH, Kingma DW, Raffeld M, et al. Association of lymphomatoid granulomatosis with Epstein-Barr viral infection of B lymphocytes and response to interferon-alpha 2b. Blood, 1996, 87(11): 4531-4537.
18. Jung KH, Sung HJ, Lee JH, et al. A case of pulmonary lymphomatoid granulomatosis successfully treated by combination chemotherapy with rituximab. Chemotherapy, 2009, 55(5): 386-390.
19. Fauci AS, Haynes BF, Costa J, et al. Lymphomatoid granulomatosis. Prospective clinical and therapeutic experience over 10 years. N Engl J Med, 1982, 306(2): 68-74.
20. Katzenstein AL, Carrington CB, Liebow AA. Lymphomatoid granulomatosis: a clinicopathologic study of 152 cases. Cancer, 1979, 43(1): 360.
21. Ankita G, Shashi D. Pulmonary lymphomatoid granulomatosis - a case report with review of literature. Indian J Surg Oncol, 2016, 7(4): 484-487.
22. Gon?alves A, Wen X, Campainha S, et al. Lymphomatoid granulomatosis - a rare pulmonary lymphoproliferative disease. Rev Port Pneumol, 2016, 22(4): 248-249.
23. Jonathan H. Chung, Jo-Anne O. Shepard, et al Lymphomatoid granulomatosis: CT and FDG-PET findings. Korean J Radiol, 2011, 12(6): 671-678.
24. 繆偉, 朱華淵, 徐衛. 世界衛生組織 2016 年淋巴瘤分類更新解讀. 中國實用內科雜志, 2016, 36(8): 647-653.
25. Song JY, Pittaluga S, Dunleavy K, et al. Lymphomatoid granulomatosis-a single institute experience: pathologic findings and clinical correlations. Am J Surg Pathol, 2015, 39(2): 141-156.
26. Borie R, Wislez M, Antoine M, et al. Lymphoproliferative disorders of the lung. Respiration, 2017, 94(2): 157-175.

Chinese Journal of Respiratory and Critical Care Medicine

A case report of pulmonary lymphomatoid granulomatosis confirmed by autopsy and review of the literature

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