Primary pulmonary lymphoma
Primary pulmonary lymphoma (PPL) refers to a clonal lymphoid proliferation affecting the lungs without any detectable extrapulmonary involvement. It is a much rarer type of pulmonary lymphoma and is most frequently represented by lymphoma of B cell lineage - often marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type.
The diagnosis of primary pulmonary lymphoma requires the following criteria at the time of initial writing (December 2012) 6.
- involvement of lung, bronchus or both are involved without evidence of mediastinal adenopathy or a mass on a chest radiograph
- no previous extra-thoracic lymphoma
- no evidence of extra-thoracic lymphoma or lymphatic leukaemia at the time of diagnosis
- there is no disease outside of the thorax for least three months following initial diagnosis within the lung
When the lung is the principal site of lymphoma, the definition also includes
- multifocal mucosa-associated lymphoid tissue (MALT) non Hodgkin lymphoma
- pulmonary involvement with satellite nodes (hilar or mediastinal)
- multiorgan involvement by lymphomatoid granulomatosis, the clonal nature of which is controversial.
Primary pulmonary lymphoma is a very rare entity and is thought to only represent only 3-4% of extranodal non Hodgkin lymphoma, <1% of a all non Hodgkin lymphoma, 0.5-1% of all primary pulmonary malignancies 1-6.
Treatment and prognosis
This entity of lymphoma is thought to carry a comparatively good prognosis 4-5.
- 1. Cadranel J, Wislez M, Antoine M. Primary pulmonary lymphoma. Eur. Respir. J. 2003;20 (3): 750-62. Pubmed citation
- 2. Graham BB, Mathisen DJ, Mark EJ et-al. Primary pulmonary lymphoma. Ann. Thorac. Surg. 2005;80 (4): 1248-53. doi:10.1016/j.athoracsur.2005.04.014 - Pubmed citation
- 3. Lee KS, Kim Y, Primack SL. Imaging of pulmonary lymphomas. AJR Am J Roentgenol. 1997;168 (2): 339-45. AJR Am J Roentgenol (citation) - Pubmed citation
- 4. Kim JH, Lee SH, Park J et-al. Primary pulmonary non-Hodgkin's lymphoma. Jpn. J. Clin. Oncol. 2004;34 (9): 510-4. Pubmed citation
- 5. Ferraro P, Trastek VF, Adlakha H et-al. Primary non-Hodgkin's lymphoma of the lung. Ann. Thorac. Surg. 2000;69 (4): 993-7. Pubmed citation
- 6. Shin CH, Paik SH, Park JS et-al. Primary pulmonary T-cell lymphoma: a case report. Korean J Radiol. 01;11 (2): 234-8. doi:10.3348/kjr.2010.11.2.234 - Free text at pubmed - Pubmed citation
- 7. Lange S. Radiology of Chest Diseases. Thieme Medical Publishers. (1998) ISBN:1588904474. Read it at Google Books - Find it at Amazon
- 8. Hare SS, Souza CA, Bain G et-al. The radiological spectrum of pulmonary lymphoproliferative disease. Br J Radiol. 2012;85 (1015): 848-64. Br J Radiol (full text) - doi:10.1259/bjr/16420165 - Free text at pubmed - Pubmed citation
Synonyms & Alternative Spellings
|Synonyms or Alternative Spelling||Include in Listings?|
|Primary pulmonary lymphoma (PPL)||✗|
|Primary lung lymphoma||✗|
|Primary non-Hodgkin's lymphoma of the lung||✗|
|Primary non-Hodgkin's lymphoma of lung||✓|