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 [2], Sowminya Arikapudi, M.B,B.S. [3]; Grammar Reviewer: Natalie Harpenau, B.S.[4]
Synonyms and keywords: Body cavity lymphoma; PEL
Overview
Primary effusion lymphoma (PEL) is rare sub-type 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. It occurs 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 and males are more commonly affected with PEL than females. Symptoms may include fever, fatigue, weight loss, night sweats, painless swellings in the neck, axilla, groin, thorax, abdomen, chest pain, abdomen pain, bone pain, and skin rash. A lymph node biopsy is generally used in diagnosis of primary effusion lymphoma and 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 described in 1989 by DM Knowles and his group.[1]
- In 1995, Cesarman et al was the first scientist to identify the association between KSHV DNA sequences within a distinct sub-type 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
- Primary effusion lymphoma is associated with human herpes virus 8 (HHV8) infection and Epstein-Barr virus (EBV) infection.[4][5]
- Among a very few of patients with human herpes virus 8 (HHV8) not associated with an effusion (a solid variant of PEL).[6]
- Primary effusion lymphoma most often occurs in immunodeficient patients such as those with HIV/AIDS. It can sometimes occur in people who have had organ transplants.[7]
- 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.[8]
- There were immunoblastic, plasmablastic and anaplastic variants with bizarre, pleomorphic nuclei.[9]
Causes
- There are no established causes for primary effusion lymphoma.
- PEL is always associated with HHV-8, also known as Kaposi sarcoma associated herpes.[4][5][8]
- PEL is most commonly present in immunodeficient patients, especially those with advanced AIDS.[4][5][8]
- An infection with Epstein-Barr virus (EBV) is also present in the majority of primary effusion lymphoma (PEL) cases.[10]
Differentiating primary effusion lymphoma from other Diseases
- Plasmablastic lymphoma (PBL)
- Burkitt lymphoma
- Pyothorax associated lymphoma (PAL)
- Anaplastic large cell lymphoma (ALCL)
- Diffuse large B-cell lymphoma (DLBCL)
Epidemiology and Demographics
- Primary effusion lymphoma is usually diagnosed among HIV infection. [13]
- Primary effusion lymphoma usually has same age and race of HIV patients.[13]
There is no racial predilection to Primary effusion lymphoma.
Incidence
- The prevalence of primary effusion lymphoma is unknown.
- Primary effusion lymphoma accounts for less than 1% of non-AIDS related lymphomas and 4% of lymphomas associated with AIDS.[11]
Age
Gender
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]
- Male
- Age > 60 years
- Systemic disease
- Smoking
- Radiation and industrial chemicals
- Chemotherapy
Screening
There is insufficient evidence to recommend routine screening for primary effusion lymphoma.
Natural History, Complications, and Prognosis
- Primary effusion lymphoma is a very fast-growing (aggressive) lymphoma.[18]
- They are usually confined to pleural, pericardial, peritoneal cavities, presenting as serous effusions without detectable tumor masses.[11]
- Prognosis is generally poor with average life expectancy of 3-4 months after diagnosis.[19]
Diagnosis
- Among the patients who present with clinical signs of PEL usually have HIV infection or Kaposi's sarcoma or Castleman's disease.[20]
- Primary effusion lymphoma diagnosed by pathological analysis of involved tissue .[20]
- WHO describe primary effusion lymphoma (PEL) cells bridge those of large cell immunoblastic lymphoma, cells with basophilic cytoplasm, large round to irregular nuclei, prominent nucleoli.
- CD Markers that found CD45 but not usually lymphocyte markers such as CD19, CD20, CD79a, CD3, CD4, or CD8. [11][21]
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]
| 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
Physical Examination
- Physical examination of primary effusion lymphoma may be remarkable for:[11]
- Fever
- Skin rash
- Tachypnea
- Cervical lymphadenopathy
- Thoracic masses suggestive of central lymphadenopathy
- Abdominal masses suggestive of central lymphadenopathy
- Peripheral lymphadenopathy
- Lower extremity edema
Laboratory Findings
- There are no specific laboratory findings associated with primary effusion lymphoma.
- PEL cells typically express a hematolymphoid marker, CD45.[11][24][25][20]
- A lymph node biopsy is diagnostic of primary effusion lymphoma.[26]
- Other laboratory findings consistent with the diagnosis of primary effusion lymphoma include complete blood count, blood chemistry studies, cytogenetic analysis, flow cytometry, immunohistochemistry, and immunophenotyping.
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 Primary effusion lymphoma.
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
- Primary effusion lymphoma may also be diagnosed using bone marrow aspiration and biopsy.[28]
Treatment
Medical Therapy
- The mainstay of therapy for primary effusion lymphoma is chemotherapy and antiretroviral therapy.[4][29]
- Drug regimen: CHOP – Cyclophosphamide AND Doxorubicin AND Vincristine AND Prednisone
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
- ↑ 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.
- ↑ 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. doi:10.1056/NEJM199505043321802. PMID 7700311.
- ↑ 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.
- ↑ 4.0 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
- ↑ 5.0 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. doi:10.1051/medsci/20183401017. PMID 29384100.
- ↑ 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. doi:10.1155/2018/9368451. ISSN 2090-6560.
- ↑ Ahmed, Omar; Veeraraghavan, Srihari (2016). "Primary Effusion Lymphoma in Solid Organ Transplant Recipient". Chest. 150 (4): 758A. doi:10.1016/j.chest.2016.08.853. ISSN 0012-3692.
- ↑ 8.0 8.1 8.2 Narkhede M, Arora S, Ujjani C (2018). "Primary effusion lymphoma: current perspectives". Onco Targets Ther. 11: 3747–3754. doi:10.2147/OTT.S167392. PMC 6029609. PMID 29988764.
- ↑ Primary effusion lymphona. BioMed Central. http://jmedicalcasereports.biomedcentral.com/articles/10.1186/1752-1947-5-60. Accessed on March 23, 2016
- ↑ Okada S, Goto H, Yotsumoto M (August 2014). "Current status of treatment for primary effusion lymphoma". Intractable Rare Dis Res. 3 (3): 65–74. doi:10.5582/irdr.2014.01010. PMC 4214239. PMID 25364646.
- ↑ 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. doi:10.2147/OTT.S167392. PMC 6029609. PMID 29988764.
- ↑ Patel, Sanjay; Xiao, Philip (2013). "Primary Effusion Lymphoma". Archives of Pathology & Laboratory Medicine. 137 (8): 1152–1154. doi:10.5858/arpa.2012-0294-RS. ISSN 0003-9985.
- ↑ 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.
- ↑ 14.0 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
- ↑ 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. doi:10.1038/bcj.2014.6. PMC 3972705. PMID 24608734.
- ↑ 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. doi:10.1155/2014/436821. ISSN 2090-6706.
- ↑ 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. doi:10.1136/jcp.2005.031807. PMC 1860519. PMID 17071811.
- ↑ 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. doi:10.1038/bcj.2014.6. ISSN 2044-5385.
- ↑ 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. doi:10.1155/2013/292301. PMID 23401819.
- ↑ 20.0 20.1 20.2 Chen, Y.-B.; Rahemtullah, A.; Hochberg, E. (2007). "Primary Effusion Lymphoma". The Oncologist. 12 (5): 569–576. doi:10.1634/theoncologist.12-5-569. ISSN 1083-7159.
- ↑ 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 2626366. PMID 19169406.
- ↑ 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. doi:10.1200/JCO.2013.54.8800. ISSN 1527-7755. PMID 25113753.
- ↑ 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. doi:10.1155/2013/292301. PMID 23401819.
- ↑ 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. doi:10.1111/j.1365-2141.2008.07057.x. PMID 18341641. Unknown parameter
|month=ignored (help) - ↑ 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. doi:10.1155/2013/292301. PMID 23401819.
- ↑ 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 2626366. PMID 19169406.
- ↑ Buchpiguel CA (2011). "Current status of PET/CT in the diagnosis and follow up of lymphomas". Rev Bras Hematol Hemoter. 33 (2): 140–7. doi:10.5581/1516-8484.20110035. PMC 3520639. PMID 23284262.
- ↑ 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. doi:10.1590/S1806-37132005000600017. ISSN 1806-3713.
- ↑ Okada, Seiji; Goto, Hiroki; Yotsumoto, Mihoko (2014). "Current status of treatment for primary effusion lymphoma". Intractable & Rare Diseases Research. 3 (3): 65–74. doi:10.5582/irdr.2014.01010. ISSN 2186-3644.