Synonyms
Body cavity-based lymphoma; PAL; PEL; PPL; Primary effusion lymphoma; Pyothorax-associated lymphoma
Definition
Secondary involvement of the pleura by systemic lymphoma is more common than primary lymphomas arising in the pleura. It is generally accepted that two distinct forms of primary pleural lymphoma (PPL) exist: primary effusion lymphoma (PEL) and pyothorax-associated lymphoma (PAL).
Primary effusion lymphoma (PEL), also termed body cavity-based lymphoma, is a large B-cell non-Hodgkin lymphoma that often presents with serous effusions and no detectable mass lesions and carries strong associations with human herpes virus 8(HHV8)/Kaposi sarcoma herpes virus (KSHV) and human immunodeficiency virus (HIV). Immunodeficiency is a hallmark association of PEL, regardless of the underlying etiology of the immunodeficiency, though a majority of cases arise specifically in association with HIV infection (Patel and Xiao 2013). However, cases have been reported in HIV-negative...
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References and Further Reading
Hirai, S., Hamanaka, Y., Mitsui, N., Morifuji, K., & Sutoh, M. (2004). Primary malignant lymphoma arising in the pleura without preceding long-standing pyothorax. Annals of Thoracic and Cardiovascular Surgery, 10, 297–300.
Nakatsuka, S., Yao, M., Hoshida, Y., Yamamato, S., Iuchi, K., & Aozasa, K. (2002). Pyothorax-associated lymphoma: A review of 106 cases. Journal of Clinical Oncology, 20, 4253–4260.
Patel, S., & Xiao, P. (2013). Primary effusion lymphoma. Archives of Pathology and Laboratory Medicine, 137, 1152–1154.
Ravikumar, G., Tirumalae, R., & Das, K. (2012). Primary pleural non-Hodgkin lymphoma in a child – an exceedingly rare disease. Journal of Pediatric Surgery, 47, E29–E31.
Vega, F., Padula, A., Valbuena, J. R., Stancu, M., Jones, D., & Medeiros, L. J. (2006). Lymphomas involving the pleura: A clinicopathologic study of 34 cases diagnosed by pleural biopsy. Archives of Pathology and Laboratory Medicine, 130, 1497–1502.
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Romano, R., Allen, T.C. (2018). Primary Pleural Lymphoma. In: Allen, T.C., Suster, S. (eds) Pathology of the Pleura and Mediastinum. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-66796-6_382
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DOI: https://doi.org/10.1007/978-3-319-66796-6_382
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