Primary effusion lymphoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sogand Goudarzi, MD, Sowminya Arikapudi, M.B,B.S. [2]

Synonyms and Keywords: Body cavity lymphoma; PEL

Overview

Primary effusion lymphoma (PEL) is rare subtype of diffuse large B-cell lymphoma (DLBCL). Primary effusion lymphoma is a very fast-growing (aggressive) lymphoma usually confined to pleural, pericardial, peritoneal body cavities, presenting as serous effusions without detectable tumor masses, occurring primarily but not exclusively in HIV-infected patients. Lymphoma cells are found in the fluid in these body cavities. Primary effusion lymphoma is associated with human herpes virus 8 (HHV8) infection and Epstein-Barr virus (EBV) infection. On microscopic histopathological analysis, neoplastic proliferation of large lymphoid cells with round to irregular nuclei, prominent nucleoli, and varying amounts of vacuolated cytoplasm are characteristic findings of primary effusion lymphoma. Primary effusion lymphoma is more commonly observed among young or middle aged patients. Males are more commonly affected with primary effusion lymphoma than females. Symptoms of primary effusion lymphoma may include fever, fatigue, weight loss, night sweats, painless swellings in the neck, axilla, groin, thorax, and abdomen, chest pain, abdomen pain, bones pain, and skin rash. A lymph node biopsy is diagnostic of primary effusion lymphoma. The mainstay of therapy for primary effusion lymphoma is chemotherapy and antiretroviral therapy.

Historical Perspective

  • The association between AIDS, Epstein-Barr virus (EBV) and primary effusion lymphoma (PEL) was describe in 1989 by DM Knowles and his group.[1]
  • In 1995, Cesarman et al was the first to identified the association between KSHV DNA sequences within a distinct subtype of AIDS and the development of primary effusion lymphoma (PEL).[2]
  • primary effusion lymphoma (PEL) was designated by Nador et al that is associated with HHV-8/KSHV, in1996.[3]

Classification

There is no established system for the classification of primary effusion lymphoma.

Pathophysiology

Causes

Differentiating primary effusion lymphoma from other Diseases

  • Primary effusion lymphoma must be differentiated from other diseases such as:[11][12]

Epidemiology and Demographics

  • Primary effusion lymphoma is usually diagnosed among HIV infection. [13]
  • Primary effusion lymphoma usually has same age, race of HIV patients.[13]

There is no racial predilection to Primary effusion lymphoma.

Incidence

  • The prevalence of primary effusion lymphoma is unknown.

Age

  • Primary effusion lymphoma is more commonly observed among young or middle aged patients.[11][14]

Gender

  • Males are more commonly affected with primary effusion lymphoma than female (6:1).[11][14]

Risk Factors

The most potent risk factor in the development of primary effusion lymphoma is human herpes virus (HHV) infection. Other risk factors include Epstein-Barr virus (EBV) infection, immunodeficient patients, and HHV-8 infection.

Less common risk factors in the development of primary effusion lymphoma include:[11][15][16][17]

Screening

There is insufficient evidence to recommend routine screening for primary effusion lymphoma.

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

There are no established criteria for the diagnosis of Primary effusion lymphoma.

Staging

Staging for primary effusion lymphoma is provided in the following table:[22]

Revised staging system for primary nodal lymphomas (Lugano classification)
Stage Involvement Extranodal (E) status
Limited
Stage I One node or a group of adjacent nodes Single extranodal lesions without nodal involvement
Stage II Two or more nodal groups on the same side of the diaphragm Stage I or II by nodal extent with limited contiguous extranodal involvement
Stage II bulky II as above with "bulky" disease Not applicable
Advanced
Stage III Nodes on both sides of the diaphragm; nodes above the diaphragm with spleen involvement Not applicable
Stage IV Additional noncontiguous extralymphatic involvement Not applicable

History and Symptoms

  • Symptoms of primary effusion lymphoma may include the following:[11][14][23]

Physical Examination

Laboratory Findings

Electrocardiogram

There are no ECG findings associated with Primary effusion lymphoma .

X-ray

There are no x-ray findings associated with Primary effusion lymphoma.

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with [disease name].

CT scan

There are no CT scan findings associated with Primary effusion lymphoma.

MRI

There are no MRI findings associated with Primary effusion lymphoma.

Other Imaging Findings

  • There are no specific imaging study associated with primary effusion lymphoma.
  • CT, MRI, and PET scan may be helpful in the diagnosis of primary effusion lymphoma.[27]

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Surgical intervention is not recommended for the management of primary effusion lymphoma.

Primary Prevention

There are no established measures for the primary prevention of primary effusion lymphoma.

Secondary Prevention

There are no established measures for the secondary prevention of primary effusion lymphoma.

References

  1. Knowles DM, Inghirami G, Ubriaco A, Dalla-Favera R (February 1989). "Molecular genetic analysis of three AIDS-associated neoplasms of uncertain lineage demonstrates their B-cell derivation and the possible pathogenetic role of the Epstein-Barr virus". Blood. 73 (3): 792–9. PMID 2537119. 
  2. Cesarman E, Chang Y, Moore PS, Said JW, Knowles DM (May 1995). "Kaposi's sarcoma-associated herpesvirus-like DNA sequences in AIDS-related body-cavity-based lymphomas". N. Engl. J. Med. 332 (18): 1186–91. PMID 7700311. doi:10.1056/NEJM199505043321802. 
  3. Nador RG, Cesarman E, Chadburn A, Dawson DB, Ansari MQ, Sald J, Knowles DM (July 1996). "Primary effusion lymphoma: a distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus". Blood. 88 (2): 645–56. PMID 8695812. 
  4. 4.0 4.1 4.2 4.3 Primary effusion lymphona. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/primary-effusion-lymphoma/?region=nb. Accessed on March 23, 2016
  5. 5.0 5.1 5.2 Gruffat H, Manet E (January 2018). "[EBV/KSHV co-infection: an effective partnership]". Med Sci (Paris) (in French). 34 (1): 79–82. PMID 29384100. doi:10.1051/medsci/20183401017. 
  6. Hashmi, Hamza; Murray, Drew; Al-Quran, Samer; Tse, William (2018). "Primary Effusion Lymphoma without an Effusion: A Rare Case of Solid Extracavitary Variant of Primary Effusion Lymphoma in an HIV-Positive Patient". Case Reports in Hematology. 2018: 1–5. ISSN 2090-6560. doi:10.1155/2018/9368451. 
  7. Ahmed, Omar; Veeraraghavan, Srihari (2016). "Primary Effusion Lymphoma in Solid Organ Transplant Recipient". Chest. 150 (4): 758A. ISSN 0012-3692. doi:10.1016/j.chest.2016.08.853. 
  8. 8.0 8.1 8.2 Narkhede M, Arora S, Ujjani C (2018). "Primary effusion lymphoma: current perspectives". Onco Targets Ther. 11: 3747–3754. PMC 6029609Freely accessible . PMID 29988764. doi:10.2147/OTT.S167392. 
  9. Primary effusion lymphona. BioMed Central. http://jmedicalcasereports.biomedcentral.com/articles/10.1186/1752-1947-5-60. Accessed on March 23, 2016
  10. Okada S, Goto H, Yotsumoto M (August 2014). "Current status of treatment for primary effusion lymphoma". Intractable Rare Dis Res. 3 (3): 65–74. PMC 4214239Freely accessible. PMID 25364646. doi:10.5582/irdr.2014.01010. 
  11. 11.0 11.1 11.2 11.3 11.4 11.5 11.6 11.7 11.8 11.9 Narkhede M, Arora S, Ujjani C (2018). "Primary effusion lymphoma: current perspectives". Onco Targets Ther. 11: 3747–3754. PMC 6029609Freely accessible . PMID 29988764. doi:10.2147/OTT.S167392. 
  12. Patel, Sanjay; Xiao, Philip (2013). "Primary Effusion Lymphoma". Archives of Pathology & Laboratory Medicine. 137 (8): 1152–1154. ISSN 0003-9985. doi:10.5858/arpa.2012-0294-RS. 
  13. 13.0 13.1 Mbulaiteye SM, Biggar RJ, Goedert JJ, Engels EA (April 2002). "Pleural and peritoneal lymphoma among people with AIDS in the United States". J. Acquir. Immune Defic. Syndr. 29 (4): 418–21. PMID 11917248. 
  14. 14.0 14.1 14.2 Primary effusion lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf57e3e27c3994bd5378/. Accessed on March 23, 2016
  15. Antar A, El Hajj H, Jabbour M, Khalifeh I, El-Merhi F, Mahfouz R, Bazarbachi A (March 2014). "Primary effusion lymphoma in an elderly patient effectively treated by lenalidomide: case report and review of literature". Blood Cancer J. 4: e190. PMC 3972705Freely accessible. PMID 24608734. doi:10.1038/bcj.2014.6. 
  16. Mohammad, Farhan; Siddique, Muhammad Neaman; Siddiqui, Faraz; Popalzai, M.; Asgari, Masoud; Odaimi, Marcel (2014). "A Unique Case of Malignant Pleuropericardial Effusion: HHV-8-Unrelated PEL-Like Lymphoma—A Case Report and Review of the Literature". Case Reports in Oncological Medicine. 2014: 1–5. ISSN 2090-6706. doi:10.1155/2014/436821. 
  17. Inoue S, Miyamoto T, Yoshino T, Yamadori I, Hagari Y, Yamamoto O (November 2006). "Primary effusion lymphoma with skin involvement". J. Clin. Pathol. 59 (11): 1221–2. PMC 1860519Freely accessible. PMID 17071811. doi:10.1136/jcp.2005.031807. 
  18. Antar, A; El Hajj, H; Jabbour, M; Khalifeh, I; EL-Merhi, F; Mahfouz, R; Bazarbachi, A (2014). "Primary effusion lymphoma in an elderly patient effectively treated by lenalidomide: case report and review of literature". Blood Cancer Journal. 4 (3): e190–e190. ISSN 2044-5385. doi:10.1038/bcj.2014.6. 
  19. Neeraj Saini, Ephraim P. Hochberg, Erica A. Linden, Smita Jha, Heinz K. Grohs & Aliyah R. Sohani (2013). "HHV8-Negative Primary Effusion Lymphoma of B-Cell Lineage: Two Cases and a Comprehensive Review of the Literature". Case reports in oncological medicine. 2013: 292301. PMID 23401819. doi:10.1155/2013/292301. 
  20. 20.0 20.1 20.2 Chen, Y.-B.; Rahemtullah, A.; Hochberg, E. (2007). "Primary Effusion Lymphoma". The Oncologist. 12 (5): 569–576. ISSN 1083-7159. doi:10.1634/theoncologist.12-5-569. 
  21. Nemunaitis MC, Schussler JM, Shiller SM, Sloan LM, Mennel RG (January 2009). "Primary effusion lymphoma diagnosed by pericardiocentesis". Proc (Bayl Univ Med Cent). 22 (1): 77–80. PMC 2626366Freely accessible. PMID 19169406. 
  22. Cheson, Bruce D.; Fisher, Richard I.; Barrington, Sally F.; Cavalli, Franco; Schwartz, Lawrence H.; Zucca, Emanuele; Lister, T. Andrew; Alliance, Australasian Leukaemia and Lymphoma Group; Eastern Cooperative Oncology Group; European Mantle Cell Lymphoma Consortium; Italian Lymphoma Foundation; European Organisation for Research; Treatment of Cancer/Dutch Hemato-Oncology Group; Grupo Español de Médula Ósea; German High-Grade Lymphoma Study Group; German Hodgkin's Study Group; Japanese Lymphorra Study Group; Lymphoma Study Association; NCIC Clinical Trials Group; Nordic Lymphoma Study Group; Southwest Oncology Group; United Kingdom National Cancer Research Institute (2014-09-20). "Recommendations for initial evaluation, staging, and response assessment of Hodgkin and non-Hodgkin lymphoma: the Lugano classification". Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology. 32 (27): 3059–3068. ISSN 1527-7755. PMID 25113753. doi:10.1200/JCO.2013.54.8800. 
  23. Neeraj Saini, Ephraim P. Hochberg, Erica A. Linden, Smita Jha, Heinz K. Grohs & Aliyah R. Sohani (2013). "HHV8-Negative Primary Effusion Lymphoma of B-Cell Lineage: Two Cases and a Comprehensive Review of the Literature". Case reports in oncological medicine. 2013: 292301. PMID 23401819. doi:10.1155/2013/292301. 
  24. Emmanuelle Boulanger, Veronique Meignin & Eric Oksenhendler (2008). "Bortezomib (PS-341) in patients with human herpesvirus 8-associated primary effusion lymphoma". British journal of haematology. 141 (4): 559–561. PMID 18341641. doi:10.1111/j.1365-2141.2008.07057.x. 
  25. Neeraj Saini, Ephraim P. Hochberg, Erica A. Linden, Smita Jha, Heinz K. Grohs & Aliyah R. Sohani (2013). "HHV8-Negative Primary Effusion Lymphoma of B-Cell Lineage: Two Cases and a Comprehensive Review of the Literature". Case reports in oncological medicine. 2013: 292301. PMID 23401819. doi:10.1155/2013/292301. 
  26. Nemunaitis MC, Schussler JM, Shiller SM, Sloan LM, Mennel RG (January 2009). "Primary effusion lymphoma diagnosed by pericardiocentesis". Proc (Bayl Univ Med Cent). 22 (1): 77–80. PMC 2626366Freely accessible. PMID 19169406. 
  27. Buchpiguel CA (2011). "Current status of PET/CT in the diagnosis and follow up of lymphomas". Rev Bras Hematol Hemoter. 33 (2): 140–7. PMC 3520639Freely accessible. PMID 23284262. doi:10.5581/1516-8484.20110035. 
  28. Antonangelo, Leila; Vargas, Francisco S; Teixeira, Lisete Ribeiro; Vaz, Marcelo A C; Sales, Maria Mirtes; Moreira, Luis C; Sales, Roberta Karla Barbosa de (2005). "Linfoma primário de cavidade pleural em paciente imunocompetente". Jornal Brasileiro de Pneumologia. 31 (6): 563–566. ISSN 1806-3713. doi:10.1590/S1806-37132005000600017. 
  29. Okada, Seiji; Goto, Hiroki; Yotsumoto, Mihoko (2014). "Current status of treatment for primary effusion lymphoma". Intractable & Rare Diseases Research. 3 (3): 65–74. ISSN 2186-3644. doi:10.5582/irdr.2014.01010. 

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