Skip to main content

Lymphoproliferative Lung Disorders

  • Chapter
  • First Online:
Orphan Lung Diseases

Abstract

In the lung, primary lymphoproliferative disease represents a wide and overlapping spectrum of conditions from reactive polymorphous and polyclonal processes through to various entities of malignant lymphoma (Table 39.1) [1, 2]. The natural history of many of the conditions is variable with further heterogeneity recognized within distinct disease entities. Primary pulmonary lymphoproliferative diseases are rare, whereas secondary pulmonary lymphoma occurs in up to 20.5% of autopsy cases. The lung is a potential organ for tumor deposition in disseminated haemato-lymphoid disease.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 189.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 169.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 249.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Poletti V, Colella S, Piciucchi S, et al. Haematological disorders and bone marrow transplant recipients. In: Wuyts WA, Cottin V, Spagnolo P, Wells AU, editors. Pulmonary manifesations of systemic diseases. (ERS monograph); 2019. p. 333–58.

    Chapter  Google Scholar 

  2. Swerdlow SH, Campo E, Harris NL, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon: IARC; 2017.

    Google Scholar 

  3. Fishback N, Koss M. Update on lymphoid interstitial pneumonitis. Curr Opin Pulm Med. 1996;2:429–533.

    Article  CAS  PubMed  Google Scholar 

  4. Escalon JG, Richards JC, Koelsch T, Downey GP, Lynch DA. Isolated cystic lung disease: an algorithmic approach to distinguishing Birt-Hogg-Dubè syndrome, lymphangioleiomyomatosis and lymphocytic interstitial pneumonia. AJR. 2019;212:1260–4.

    Article  PubMed  Google Scholar 

  5. Shimakage M, Sakamoto H, Harada S, et al. Expression of the Epstein-Barr virus in lymphoproliferative diseases of the lung. Oncol Rep. 2007;17:1347–52.

    PubMed  Google Scholar 

  6. Bhoopat L, Rangkakulnuwat S, Ya-In C, et al. Relationship of cell bearing EBER and p24 antigens in biopsy-proven lymphocytic interstitial pneumonia in HIV-1 subtype E infected children. Appl Immunohistochem Mol Morphol. 2011;19:547–51.

    Article  CAS  PubMed  Google Scholar 

  7. Yousem SA, Colby TV, Carrington CB. Follicular bronchitis/bronchiolitis. Hum Pathol. 1985;16:700–6.

    Article  CAS  PubMed  Google Scholar 

  8. Castleman B, Iverson L, Menendez VP. Localized mediastinal lymphnode hyperplasia resembling thymoma. Cancer. 1956;9:822–30.

    Article  CAS  PubMed  Google Scholar 

  9. Wu D, Lim MS, Jaffe ES. Pathology of Castleman disease. Hematol Oncol Clin N Am. 2018;32:37–52.

    Article  Google Scholar 

  10. Igawa T, Sato Y. TAFRO syndrome. Hematol Oncol Clin North Am. 2018;32:107–18.

    Article  PubMed  Google Scholar 

  11. Oksenhendler E. Maladies de Castleman. In: Guillevin L, Meyer O, Hachulla E, et al., editors. Traité de Maladies et syndromes systematiques. 6th ed. Paris: Lavoisier Medicine Sciences; 2015. p. 1064–76.

    Google Scholar 

  12. Terasaki Y, Ikushima S, Matsui S, et al. Comparison of clinical and pathological features of lung lesions of systemic IgG4-related disease and idiopathic multicentric Castleman’s disease. Histopathology. 2017;70:1114–24.

    Article  PubMed  Google Scholar 

  13. Borie R, Cadranel J, Guihot A, et al. Pulmonary manifestations of human herpesvirus-8 during HIV infection. Eur Respir J. 2013;42:1105–18.

    Article  PubMed  Google Scholar 

  14. Fajgenbaum DC. Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease. Blood. 2018;132:2323–30.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Van Rhee F, Voorhees P, Dispenzieri A, et al. International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease. Blood. 2018;132:2115–24.

    Article  PubMed  PubMed Central  Google Scholar 

  16. Ahmed S, Kussick SJ, Siddiqui AK, et al. Bronchial-associated lymphoid tissue lymphoma: a clinical study of a rare disease. Eur J Cancer. 2004;40:1320–6.

    Article  PubMed  Google Scholar 

  17. Adam P, Czapiewski P, Colak S, et al. Prevalence of Achromobacter xyloxidans in pulmonary mucosa associated lymphoid tissue lymphoma in different regions of Europe. Br J Haematol. 2014;164:804–10.

    Article  CAS  PubMed  Google Scholar 

  18. Cordier J, Chailleux E, Lauque D, et al. Primary pulmonary lymphomas. A clinical study of 70 cases in non immunocompromised patients. Chest. 1993;103:201–8.

    Article  CAS  PubMed  Google Scholar 

  19. Borie R, Wislez M, Thabut G, et al. Clinical characteristics and prognostic factors of pulmonary MALT lymphoma. Eur Respir J. 2009;34:1408–16.

    Article  CAS  PubMed  Google Scholar 

  20. Isaacson PG, Du MQ. MALT lymphoma: from morphology to molecules. Nat Rev Cancer. 2004;4:644–453.

    Article  CAS  PubMed  Google Scholar 

  21. Zucca E, Bertoni F. The spectrum of MALT lymphoma at different sites. Biological and therapeutic relevance. Blood. 2016;127:2082–92.

    Article  CAS  PubMed  Google Scholar 

  22. Habermann TM, Ryu JH, Inwards DJ, et al. Primary pulmonary lymphoma. Semin Oncol. 1999;26:307–15.

    CAS  PubMed  Google Scholar 

  23. Borie R, Wislez M, Antoine M, Cadranel J. Lymphoproliferative disorders of the lung. Respiration. 2017;94:157–75.

    Article  PubMed  Google Scholar 

  24. King LJ, Padley SP, Wotherspoon AC, Nicholson AG. Pulmonary MALT lymphoma: imaging findings in 24 cases. Eur Radiol. 2000;10:1932–8.

    Article  CAS  PubMed  Google Scholar 

  25. Kurtin PJ, Myers JL, Adlakha H, et al. Pathologic and clinical fea- tures of primary pulmonary extranodal marginal zone B-cell lym- phoma of MALT type. Am J Surg Pathol. 2001;25:997–1008.

    Article  CAS  PubMed  Google Scholar 

  26. Nicholson AG, Wotherspoon AC, Diss TC, et al. Pulmonary B-cell non-Hodgkin’s lymphomas. The value of immunohistochemistry and gene analysis in diagnosis. Histopathology. 1995;26:395–403.

    Article  CAS  PubMed  Google Scholar 

  27. Shaikh G, Sehgal R, Mehrishi A, Karnik A. Primary pulmonary plasmacytoma. J Clin Oncol. 2008;26:3089–91.

    Article  PubMed  Google Scholar 

  28. Shah BK, Shrestha A. Pulmonary plasmacytoma. J Thorac Oncol. 2012;7:923.

    Article  PubMed  Google Scholar 

  29. Horiuchi T, Hirokawa M, Oyama Y, Kitabayashi A, Satoh K, Shindoh T, et al. Diffuse pulmonary infiltrates as a roentgeno—graphic manifestation of primary pulmonary plasmacytoma. Am J Med. 1998;105:72–4.

    Article  CAS  PubMed  Google Scholar 

  30. Cheah CY, Lingaratnam S, Seymour JF. Rituximab for the treatment of follicular lymphoma. Future Oncol. 2013;9:1283–98.

    Article  CAS  PubMed  Google Scholar 

  31. Morice WG, Colby TV. Lymphoproliferative lung diseases. In: Tomashefski Jr JF, editor. Dail and Hammar’s pulmonary pathology. 3rd ed. Berlin: Springer; 2015. p. 1–46.

    Google Scholar 

  32. Chilosi M, Zinzani PL, Poletti V. Lymphoproliferative lung disorders. Semin Respir Crit Care Med. 2005;26:490–501.

    Article  PubMed  Google Scholar 

  33. Li G, Hansmann ML, Zwingers T, Lennert K. Primary lymphomas of the lung: morphological, immunohistochemical and clinical features. Histopathology. 1990;16:519–31.

    Article  CAS  PubMed  Google Scholar 

  34. Poletti V, Gurioli C, Piciucchi S, Rossi A, Ravaglia C, Dubini A, Asioli CGL. Intravascular large B cell lymphoma presenting in the lung:the diagnostic value of transbronchial cryobiopsy. Sarcoidosis Vasc Diffuse Lung Dis. 2015;31:354–8.

    PubMed  Google Scholar 

  35. Lee JS, Tuder R, Lynch DA. Lymphomatoid granulomatosis: radiologic, features and pathologic correlations. Am J Roentgenol. 2000;175:1335–133.

    Article  CAS  Google Scholar 

  36. Sukswai N, Lyapichev K, Khoury JD, Medeiros LJ. Diffuse large B-cell lymphoma variants:an update. Pathology. 2020;52:53–67.

    Article  CAS  PubMed  Google Scholar 

  37. Travis WD, Brambilla E, Műller-Hermelink HK, Harris CC. WHO: pathology and genetics of tumours of the lung, pleura, thymus and heart. Lyon: IARC Press; 2004.

    Google Scholar 

  38. Bolaman Z, Kadikoylu G, Polatli M, Barutca S, Culhaci N, Senturk T. Migratory nodules in the lung: lymphomatoi granulomatosis. Leuk Lymphoma. 2003;44:197–200.

    Article  CAS  PubMed  Google Scholar 

  39. Siegloch K, Schmitz N, Wu HS, et al. Hematopoietic stem cell transplantation in patients with lymphomatoid granulomatosis: a European group for blood and marrow transplantation report. Biol Blood Marrow Transplant. 2013;19:1522–5.

    Article  PubMed  Google Scholar 

  40. Aoki T, Harada Y, Matsubara E, et al. Long-term remission after multiple relapses in an elderly patient with lymphomatoid granulomatosis after rituximab and high-dose cytarabine chemotherapy without stem-cell transplantation. J Clin Oncol. 2013;31:e390.

    Article  PubMed  Google Scholar 

  41. Shet T, Suryawanshi P, Epari S, et al. Extranodal natural killer/T cell lymphomas with extranasal disease in non-endemic regions are disseminated or have nasal primary: a study of 84 cases from India. Leuk Lymphoma. 2014;55:2748–53.

    Article  CAS  PubMed  Google Scholar 

  42. Au WY, Weisenburger DD, Intragumtornchai T, Nakamura S, Kim WS, Sng I, et al. Clinical differences between nasal and extranasal natural killer/T-cell lymphoma: a study of 136 cases from the international peripheral T-cell lymphoma project. Blood. 2009;113:3931–7.

    Article  CAS  PubMed  Google Scholar 

  43. Tse E, Kwong YL. NK/T cell lymphomas. Best Pract Res Clin Haematol. 2019;32:253–61.

    Article  PubMed  Google Scholar 

  44. Sverzellati N, Poletti V, Chilosi M, Casoni GL, Hansell DM, Zompatori M. The crazy-paving pattern in granulomatous mycosis fungoides: HRTC-pathologic correlation. J Comput Assist Tomogr. 2006;30:843–5.

    Article  PubMed  Google Scholar 

  45. Yousen SA, Weiss LM, Colby TV. Primary pulmonary Hodgkin’s disease. Cancer. 1986;57:1217–724.

    Article  Google Scholar 

  46. Radin AI. Primary pulmonary Hodgkin’s disease. Cancer. 1990;65:550–63.

    Article  CAS  PubMed  Google Scholar 

  47. Cartier Y, Johkoh T, Honda O, Müller NL. Primary pulmonary Hodgkin’s disease: CT findings in three patients. Clin Radiol. 1999;54:182–4.

    Article  CAS  PubMed  Google Scholar 

  48. Romero S, Montoro J, Guinot M, et al. Post-transplant lymphoproliferative disorders after solid organ and hematopoietic stem cell transplantation. Leuk Lymphoma. 2019;60:142–50.

    Article  PubMed  Google Scholar 

  49. Yoon GY, Kim MY, Shim TS. Posttransplant lymphoproliferative disorder of the thorax: CT and FDG-PET features in a single tertiary referral center. Medicine (Baltimore). 2015;94:e1274.

    Article  PubMed  Google Scholar 

  50. Poletti V, Chilosi M, Olivieri D. Diagnostic invasive procedures in diffuse infiltrative lung diseases. Respiration. 2004;71:107–19.

    Article  PubMed  Google Scholar 

  51. Poletti V, editor. Transbronchial cryobiopsy in diffuse parenchymal lung diseases. Berlin: Springer; 2019.

    Google Scholar 

  52. Iqbal S, Depew ZS, Kurtin PJ, et al. Endobronchial ultrasound and lymphoproliferative disorders: a retrospective study. Ann Thorac Surg. 2012;94:1830–4.

    Article  PubMed  Google Scholar 

  53. Poletti V, Romagna M, Gasponi A, Baruzzi G, Allen KA. Bronchoalveolar lavage in the diagnosis of low-grade, MALT type, B-cell lymphoma in the lung. Monaldi Arch Chest Dis. 1995;50:191–4.

    CAS  PubMed  Google Scholar 

  54. Zoie S, Couderc LJ, Cadranel J, Antoine MA, Epardeau B, Fleury-Feith J, Popa N, Santoli F, Farcet JP, Delfau-Larue MH. Clonality analysis of alveolar B lymphocytes contributes to the diagnostic strategy in clinical suspicion of pulmonary lymphoma. Blood. 2004;103:3208–15.

    Article  Google Scholar 

  55. Oh SY, Kim WS, Kim JS, et al. Multiple mucosa-associated lymphoid tissue organs involving marginal zone B cell lymphoma: organ-specifi c relationships and the prognostic factors. Consortiumfor improving survival of lymphoma study. Int J Hematol. 2010;92:510–7.

    Article  PubMed  Google Scholar 

  56. de Boer JP, Hiddink RF, Raderer M, et al. Dissemination patterns in non-gastric MALT lymphoma. Haematologica. 2008;93:201–16.

    Article  PubMed  Google Scholar 

  57. Lenz G, Pasqualucci L, editors. Malignant lymphomas: biology and molecular pathogenesis. Berlin: De Gruyter; 2016.

    Google Scholar 

  58. Zinzani PL, Casadei B, Derenzini E, et al. Extranodal marginal zone B-cell lymphoma of the lung: experience with fludarabine and mitoxantrone-containing regimens. Hematol Oncol. 2013;31:183–8.

    Article  CAS  PubMed  Google Scholar 

  59. Zinzani PL, Tura S, Cavo M, Lewis RE. Hematology:pathophysiology, diagnosis and treatment. Bologna: Esculapio; 2018.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Venerino Poletti .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2023 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Poletti, V. et al. (2023). Lymphoproliferative Lung Disorders. In: Cottin, V., Richeldi, L., Brown, K., McCormack, F.X. (eds) Orphan Lung Diseases. Springer, Cham. https://doi.org/10.1007/978-3-031-12950-6_39

Download citation

  • DOI: https://doi.org/10.1007/978-3-031-12950-6_39

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-031-12949-0

  • Online ISBN: 978-3-031-12950-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics