PRIMARY PULMONARY HODGKIN'S LYMPHOMA: A CASE OF DIAGNOSTIC COMPLEXITY AND TREATMENT RESISTANCE
Abstract
Primary pulmonary Hodgkin's lymphoma (PPHL) arises from the lymphoid tissue of the alveolar mucosa or peribronchial lymph nodes. PPHL is extremely rare, representing only 1.5–2.4% of all cases of primary pulmonary lymphoma.[1] Its rarity and nonspecific presentation often lead to diagnostic delays. Symptoms such as cough, fatigue, or chest discomfort mimic other pulmonary diseases, complicating clinical evaluation. Here, we present a case of PPHL initially misdiagnosed as granulomatosis with polyangiitis due to overlapping clinical and histopathological findings. This case emphasizes the importance of a multidisciplinary approach and highlights the critical role of thorough histopathological analysis in establishing an accurate diagnosis.
References
Jaffe ES, Harris NL, Stein H, Vardiman JW. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues. WHO; 2008.
Cadranel J, Wislez M, Antoine M. Primary pulmonary lymphomas: a clinical study of 70 cases. Chest. 1998;114(1):124-132.
Ponzoni M, Ferreri AJ, Zucca E. Extranodal lymphomas: pathophysiology and management. Haematologica. 2007;92(9):1187-1194.
Beasley MB, Franks TJ, Travis WD. Primary pulmonary lymphoma: clinical and pathologic features. Arch Pathol Lab Med. 2005;129(1):94-98.
Lichtenstein AK, Jett JR, Downie GH. Uncommon pulmonary tumors. Clin Chest Med. 2004;25(1):245-261.
Katzenstein A-LA, Mukhopadhyay S, Myers JL. Diagnostic Atlas of Non-Neoplastic Lung Disease. Springer; 2010.
Rossi G, Cavazza A, Spagnolo P, et al. Pulmonary lymphomas and lymphoid hyperplasias. Semin Respir Crit Care Med. 2011;32(4):430-441.
Godfrey RS, Antippa P, Lamb DS. Hodgkin lymphoma of the lung: an elusive diagnosis. J Thorac Oncol. 2016;11(4):623-625.
Travis WD, Brambilla E, Burke AP, Marx A, Nicholson AG. Thoracic Tumours. IARC Press; 2021.
Moskowitz CH, Nademanee A, Masszi T, et al. Brentuximab vedotin as consolidation after autologous stem-cell transplantation in patients with Hodgkin's lymphoma at risk of relapse or progression (AETHERA): a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet. 2015;385(9980):1853-1862.
Cordier JF, Loire R, Brune J. Primary pulmonary lymphomas. Chest. 1993;103(2):494-503.
Ryu JH, Colby TV, Hartman TE, Vassallo R. Diffuse Lung Disease: A Practical Approach. Springer; 2005.
Cheson BD, Fisher RI, Barrington SF, et al. Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification. J Clin Oncol. 2014;32(27):3059-3068. https://doi.org/10.1200/JCO.2013.54.8800.
Younes A, Gopal AK, Smith SE, et al. Results of a pivotal phase II study of brentuximab vedotin for patients with relapsed or refractory Hodgkin’s lymphoma. J Clin Oncol. 2012;30(18):2183-2189. https://doi.org/10.1200/JCO.2011.38.0410.
Armitage JO, Longo DL. Chemotherapy for Hodgkin lymphoma in adults: clinical practice guideline. Ann Oncol. 2019;30(7):947-960.
Barrington SF, Mikhaeel NG, Kostakoglu L, et al. Role of imaging in the staging and response assessment of lymphoma: consensus of the International Conference on Malignant Lymphomas Imaging Working Group. J Clin Oncol. 2014;32(27):3048-3058.
Engert A, Eichenauer DA, Fuchs M, Anagnostopoulos I, Diehl V. Hodgkin's lymphoma: diagnosis and treatment. Lancet. 2010;376(9743):146-155. https://doi.org/10.1016/S0140-6736(10)60603-4.
Ansell SM. Hodgkin lymphoma: 2018 update on diagnosis, risk-stratification, and management. Am J Hematol. 2018;93(5):704-715. https://doi.org/10.1002/ajh.25063.
Townsend W, Linch D. Hodgkin's lymphoma in adults. Lancet. 2012;380(9844):836-847. https://doi.org/10.1016/S0140-6736(12)60035-X.
Connors JM. State-of-the-art therapeutics: Hodgkin's lymphoma. J Clin Oncol. 2005;23(26):6400-6408. https://doi.org/10.1200/JCO.2005.05.014.
Gandhi MK, Marcus RE. The EBV-associated lymphomas. Adv Hematol. 2011;2011:325178. https://doi.org/10.1155/2011/325178.
Gallamini A, Hutchings M, Rigacci L, et al. Early interim 2-[18F]fluoro-2-deoxy-D-glucose positron emission tomography is prognostically superior to international prognostic score in advanced-stage Hodgkin’s lymphoma: a report from a joint Italian-Danish study. J Clin Oncol. 2007;25(24):3746-3752. https://doi.org/10.1200/JCO.2007.11.6525.
O’Connor OA. Brentuximab vedotin: delivering a targeted punch in relapsed/refractory Hodgkin lymphoma. Oncologist. 2012;17(7):911-912. https://doi.org/10.1634/theoncologist.2012-0207.
Diefenbach CS, Connors JM. The role of brentuximab vedotin in patients with newly diagnosed Hodgkin lymphoma. Semin Hematol. 2016;53(3):165-171. https://doi.org/10.1053/j.seminhematol.2016.05.004.
Tarella C, Ladetto M, Zanni M, et al. Hodgkin's lymphoma: the paradigm of cure and future challenges. Leukemia. 2017;31(9):2032-2043. https://doi.org/10.1038/leu.2017.174.
Armitage JO. Early-stage Hodgkin's lymphoma. N Engl J Med. 2010;363(7):653-662. https://doi.org/10.1056/NEJMcp1000250.
Bröckelmann PJ, Borchmann P, Engert A. Current and future treatment strategies for Hodgkin lymphoma. Cancer Treat Rev. 2017;55:48-60. https://doi.org/10.1016/j.ctrv.2017.02.002.
Views:
27
Downloads:
13
Copyright (c) 2025 Radosław Pastuszek, Magdalena Rosiewicz, Karolina Bieńkowska, Aleksandra Pastuszek, Anita Janda, Sylwia Bartolik, Marcin Durowicz, Iwona Górnicka, Jan Drzymała, Urszula Kierepka
This work is licensed under a Creative Commons Attribution 4.0 International License.
All articles are published in open-access and licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0). Hence, authors retain copyright to the content of the articles.
CC BY 4.0 License allows content to be copied, adapted, displayed, distributed, re-published or otherwise re-used for any purpose including for adaptation and commercial use provided the content is attributed.
