Bronchiolitis obliterans associated with lymphoma and paraneoplastic pemphigus: one case report and literature review_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

ZHANG Xiaoyan ,  LIN Jiangtao , JIA Yuping , GAN Shibao , DA Jiping , YU Mingan

Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing 100029, P. R. China;

Corresponding author：

LIN Jiangtao, Email: jiangtao_l@263.net

Keywords：

Lymphoma; Paraneoplastic pemphigus; Bronchiolitis obliterans

DOI：

10.7507/1671-6205.201709055

Video：

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Objective To explore the clinical characteristics, diagnose and treatment of bronchiolitis obliterans (BO) associated with lymphoma and paraneoplastic pemphigus (PNP). Methods One patient with BO associated with lymphoma and paraneoplastic pemphigus was presented and literatures which searched through Wanfang Med online and PubMed on this subject were reviewed. Results A 44-year-old woman was admitted to this hospital because of oral ulcer for two years, and persistent dyspnea for fourteen months. She had been diagnosed with PNP one year earlier. Pulmonary function displayed severe obstructive ventilator dysfunction, negative response to the bronchodilator. Chest CT showed bronchiolectasis. The ratio of neutrophils increased in bronchoalveolar lavage fluid. The pathology of ultrasound-guided neck and inguinal lymph node biopsy showed non-Hodgkin’s lymphoma. Based on the clinical presentation, the image and pathology of this patient, the diagnosis of having PNP with BO secondary to the lymphoma was made. After 10 cycles of CHOP chemotherapy, lymphoma and PNP went into remission, but the patient died from respiratory failure. The literatures were reviewed and 15 patients were found, out of them 60% (9/15) were female, 47% (7/15) follicular lymphoma and 47% (7/15) diagnosis of clinical BO which did not be supported by pathology. Lymphoma-PNP-BO was a clinical syndrome and 67% of the initial presentations were PNP. Patients died within 7 to 60 months. Conclusions BO associated with lymphoma and PNP is rare and the most common initial presentation is PNP. BO is the major cause of death. Treatments included chemotherapy, corticosteroid and immunosuppressor, lymphoma and PNP can be alleviated, but BO is aggravating.

Citation： ZHANG Xiaoyan, LIN Jiangtao, JIA Yuping, GAN Shibao, DA Jiping, YU Mingan. Bronchiolitis obliterans associated with lymphoma and paraneoplastic pemphigus: one case report and literature review. Chinese Journal of Respiratory and Critical Care Medicine, 2019, 18(1): 70-75. doi: 10.7507/1671-6205.201709055 Copy

1.	牟向东, 王广发, 熊焰, 等. Castleman 病和副肿瘤性天疱疮相关性闭塞性细支气管炎. 国际呼吸杂志, 2014, 34(22): 1699-1702.
2.	陈丹, 林澄昱, 韩潇, 等. 滤泡淋巴瘤合并副肿瘤性自身免疫多器官综合征一例报告并文献复习. 中华血液学杂志, 2016, 37(12): 1049-1053.
3.	Wind LJ, van der Velden AW, Diercks GF, et al. Paraneoplastic pemphigus in a patient with non-Hodgkin lymphoma. Ned Tijdschr Geneeskd, 2010, 154: A2183.
4.	Zhu X, Zhang B. Paraneoplastic pemphigus. J Dermatol, 2007, 34(8): 503-511.
5.	Hirano T, Higuchi Y, Yuki H, et al. Rituximab monotherapy and rituximab-containing chemotherapy were effective for paraneoplastic pemphigus accompanying follicular lymphoma, but not for subsequent bronchiolitis obliterans. J Clin Exp Hematop, 2015, 55(2): 83-88.
6.	Suzuki K, Nishiwaki K, Yamada H, et al. Case of paraneoplastic pemphigus associated with retroperitoneal diffuse large B-cell lymphoma and fatal bronchiolitis obliterans-like lung disease. J Dermatol, 2013, 40(2): 142-144.
7.	Nousari HC, Deterding R, Wojtczack H, et al. The mechanism of respiratory failure in paraneoplastic pemphigus. N Engl J Med, 1999, 340(18): 1406-1410.
8.	Hasegawa Y, Shimokata K, Ichiyama S, et al. Constrictive bronchiolitis obliterans and paraneoplastic pemphigus. Eur Respir J, 1999, 13(4): 934-937.
9.	Namba C, Tohyama M, Hanakawa Y, et al. Paraneoplastic pemphigus associated with fatal bronchiolitis obliterans and intractable mucosal erosions: treatment with cyclosporin in addition to steroid, rituximab and intravenous immunoglobulin. J Dermatol, 2016, 43(4): 419-422.
10.	Morikawa K, Tsuji T, Yamasaki H, et al. Paraneoplastic pemphigus occurs most commonly in indolent B cell lymphoma. Acta Haematol, 2014, 132(1): 73-74.
11.	Kanaoka M, Matsukura S, Ishikawa H, et al. Paraneoplastic pemphigus associated with fatal bronchiolitis obliterans and appearance of anti-BP180 antibodies in the late stage of the disease. J Dermatol, 2014, 41(7): 628-630.
12.	Gudi VS, Ormerod AD, Weir J, et al. Severe breathlessness, mouth ulcers and skin blistering in a female. Eur Respir J, 2004, 24(5): 884-887.
13.	Cordel N, Ringeisen F, Antoine M, et al. Paraneoplastic pemphigus with constrictive bronchiolitis obliterans. Dermatology, 2001, 202(2): 145.
14.	Takahashi M, Shimatsu Y, Kazama T, et al. Paraneoplastic pemphigus associated with bronchiolitis obliterans. Chest, 2000, 117(2): 603-607.
15.	Wang SH, Chu CY, Chen HH, et al. Paraneoplastic pemphigus and bronchiolitis obliterans in a patient with splenic B-cell lymphoma. J Formos Med Assoc, 2007, 106(9): 768-773.
16.	Maldonado F, Pittelkow MR, Ryu JH. Constrictive bronchiolitis associated with paraneoplastic autoimmune multi-organ syndrome. Respirology, 2009, 14(1): 129-133.
17.	Sanz-Bueno J, Cullen D, Zarco C, et al. Paraneoplastic autoimmune multiorgan syndrome (paraneoplastic pemphigus) with unusual manifestations and without detectable autoantibodies. Indian J Dermatol Venereol Leprol, 2014, 80(4): 328-330.
18.	Lee S, Yamauchi T, Ishii N, et al. Achievement of the longest survival of paraneoplastic pemphigus with bronchiolitis obliterans associated with follicular lymphoma using R-CHOP chemotherapy. Int J Hematol, 2017, 106(6): 852-859.
19.	陈志敏. 儿童闭塞性细支气管炎诊断和治疗进展. 实用儿科临床杂志, 2011, 26(16): 1239-1241.
20.	刘琴, 陈德晖, 曾庆思, 等. 儿童闭塞性细支气管炎 HRCT 表现. 放射学实践, 2013, 28(11): 1098-1011.
21.	Kligerman SJ, Henry T, Lin CT, et al. Mosaic attenuation: etiology, methods of differentiation, and pitfalls. Radiographics, 2015, 35(5): 1360-1380.
22.	Worthy SA, Müller NL, Hartman TE, et al. Mosaic attenuation pattern on thin-section CT scans of the lung: differentiation among infiltrative lung, airway, and vascular diseases as a cause. Radiology, 1997, 205(2): 465-470.
23.	Anhalt GJ, Kim SC, Stanley JR, et al. Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia. N Engl J Med, 1990, 323(25): 1729-1735.
24.	王爱平, 朱学骏, 涂平, 等. 副肿瘤性天疱疮二例. 中华皮肤科杂志, 2000, 33(6): 392-393.
25.	王京, 李静, 朱学骏. 副肿瘤性天疱疮的临床路径与发病机理. 岭南皮肤性病科杂志, 2009, 16(6): 347-350.
26.	Kaplan I, Hodak E, Ackerman L, et al. Neoplasms associated with paraneoplastic pemphigus: a review with emphasis on non-hematologic malignancy and oral mucosal manifestations. Oral Oncol, 2004, 144: 1102-1104.
27.	Hashimoto T. Immunopathology of paraneoplastic pemphigus. Clin Dermatol, 2001, 19: 675-682.
28.	Jain R, Hachem RR, Morrell MR, et al. Azithromycin is associated with increased survival in lung transplant recipients with bronchiolitis obliterans syndrome. J Heart Lung Transplant, 2010, 29(5): 531-537.
29.	Lynch JP 3rd, Weigt SS, DerHovanessian A, et al. Obliterative (constrictive) bronchiolitis. Semin Respir Crit Care Med, 2012, 33(5): 509-532.
30.	Hasleton PS. Spencer’s pathology of the lung. 5th Edn. New York: McGraw-Hill, 1996: 240-255.

1. 牟向东, 王广发, 熊焰, 等. Castleman 病和副肿瘤性天疱疮相关性闭塞性细支气管炎. 国际呼吸杂志, 2014, 34(22): 1699-1702.
2. 陈丹, 林澄昱, 韩潇, 等. 滤泡淋巴瘤合并副肿瘤性自身免疫多器官综合征一例报告并文献复习. 中华血液学杂志, 2016, 37(12): 1049-1053.
3. Wind LJ, van der Velden AW, Diercks GF, et al. Paraneoplastic pemphigus in a patient with non-Hodgkin lymphoma. Ned Tijdschr Geneeskd, 2010, 154: A2183.
4. Zhu X, Zhang B. Paraneoplastic pemphigus. J Dermatol, 2007, 34(8): 503-511.
5. Hirano T, Higuchi Y, Yuki H, et al. Rituximab monotherapy and rituximab-containing chemotherapy were effective for paraneoplastic pemphigus accompanying follicular lymphoma, but not for subsequent bronchiolitis obliterans. J Clin Exp Hematop, 2015, 55(2): 83-88.
6. Suzuki K, Nishiwaki K, Yamada H, et al. Case of paraneoplastic pemphigus associated with retroperitoneal diffuse large B-cell lymphoma and fatal bronchiolitis obliterans-like lung disease. J Dermatol, 2013, 40(2): 142-144.
7. Nousari HC, Deterding R, Wojtczack H, et al. The mechanism of respiratory failure in paraneoplastic pemphigus. N Engl J Med, 1999, 340(18): 1406-1410.
8. Hasegawa Y, Shimokata K, Ichiyama S, et al. Constrictive bronchiolitis obliterans and paraneoplastic pemphigus. Eur Respir J, 1999, 13(4): 934-937.
9. Namba C, Tohyama M, Hanakawa Y, et al. Paraneoplastic pemphigus associated with fatal bronchiolitis obliterans and intractable mucosal erosions: treatment with cyclosporin in addition to steroid, rituximab and intravenous immunoglobulin. J Dermatol, 2016, 43(4): 419-422.
10. Morikawa K, Tsuji T, Yamasaki H, et al. Paraneoplastic pemphigus occurs most commonly in indolent B cell lymphoma. Acta Haematol, 2014, 132(1): 73-74.
11. Kanaoka M, Matsukura S, Ishikawa H, et al. Paraneoplastic pemphigus associated with fatal bronchiolitis obliterans and appearance of anti-BP180 antibodies in the late stage of the disease. J Dermatol, 2014, 41(7): 628-630.
12. Gudi VS, Ormerod AD, Weir J, et al. Severe breathlessness, mouth ulcers and skin blistering in a female. Eur Respir J, 2004, 24(5): 884-887.
13. Cordel N, Ringeisen F, Antoine M, et al. Paraneoplastic pemphigus with constrictive bronchiolitis obliterans. Dermatology, 2001, 202(2): 145.
14. Takahashi M, Shimatsu Y, Kazama T, et al. Paraneoplastic pemphigus associated with bronchiolitis obliterans. Chest, 2000, 117(2): 603-607.
15. Wang SH, Chu CY, Chen HH, et al. Paraneoplastic pemphigus and bronchiolitis obliterans in a patient with splenic B-cell lymphoma. J Formos Med Assoc, 2007, 106(9): 768-773.
16. Maldonado F, Pittelkow MR, Ryu JH. Constrictive bronchiolitis associated with paraneoplastic autoimmune multi-organ syndrome. Respirology, 2009, 14(1): 129-133.
17. Sanz-Bueno J, Cullen D, Zarco C, et al. Paraneoplastic autoimmune multiorgan syndrome (paraneoplastic pemphigus) with unusual manifestations and without detectable autoantibodies. Indian J Dermatol Venereol Leprol, 2014, 80(4): 328-330.
18. Lee S, Yamauchi T, Ishii N, et al. Achievement of the longest survival of paraneoplastic pemphigus with bronchiolitis obliterans associated with follicular lymphoma using R-CHOP chemotherapy. Int J Hematol, 2017, 106(6): 852-859.
19. 陈志敏. 儿童闭塞性细支气管炎诊断和治疗进展. 实用儿科临床杂志, 2011, 26(16): 1239-1241.
20. 刘琴, 陈德晖, 曾庆思, 等. 儿童闭塞性细支气管炎 HRCT 表现. 放射学实践, 2013, 28(11): 1098-1011.
21. Kligerman SJ, Henry T, Lin CT, et al. Mosaic attenuation: etiology, methods of differentiation, and pitfalls. Radiographics, 2015, 35(5): 1360-1380.
22. Worthy SA, Müller NL, Hartman TE, et al. Mosaic attenuation pattern on thin-section CT scans of the lung: differentiation among infiltrative lung, airway, and vascular diseases as a cause. Radiology, 1997, 205(2): 465-470.
23. Anhalt GJ, Kim SC, Stanley JR, et al. Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia. N Engl J Med, 1990, 323(25): 1729-1735.
24. 王爱平, 朱学骏, 涂平, 等. 副肿瘤性天疱疮二例. 中华皮肤科杂志, 2000, 33(6): 392-393.
25. 王京, 李静, 朱学骏. 副肿瘤性天疱疮的临床路径与发病机理. 岭南皮肤性病科杂志, 2009, 16(6): 347-350.
26. Kaplan I, Hodak E, Ackerman L, et al. Neoplasms associated with paraneoplastic pemphigus: a review with emphasis on non-hematologic malignancy and oral mucosal manifestations. Oral Oncol, 2004, 144: 1102-1104.
27. Hashimoto T. Immunopathology of paraneoplastic pemphigus. Clin Dermatol, 2001, 19: 675-682.
28. Jain R, Hachem RR, Morrell MR, et al. Azithromycin is associated with increased survival in lung transplant recipients with bronchiolitis obliterans syndrome. J Heart Lung Transplant, 2010, 29(5): 531-537.
29. Lynch JP 3rd, Weigt SS, DerHovanessian A, et al. Obliterative (constrictive) bronchiolitis. Semin Respir Crit Care Med, 2012, 33(5): 509-532.
30. Hasleton PS. Spencer’s pathology of the lung. 5th Edn. New York: McGraw-Hill, 1996: 240-255.

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Bronchiolitis obliterans associated with lymphoma and paraneoplastic pemphigus: one case report and literature review

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