Primary effusion lymphoma
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: 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.
Pathophysiology
- Primary effusion lymphoma is associated with human herpes virus 8 (HHV8) infection and Epstein-Barr virus (EBV) infection.[1]
- 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.
- 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.
- There were immunoblastic, plasmablastic and anaplastic variants with bizarre, pleomorphic nuclei.[2]
Causes
- There are no established causes for primary effusion lymphoma.
- PEL is always associated with HHV-8, also known as Kaposi sarcoma-associated herpes.
- PEL is most commonly present in immunodeficient patients, especially those with advanced AIDS.
- An infection with Epstein-Barr virus (EBV) is also present in the majority of PEL cases.
Differentiating primary effusion lymphoma from other Diseases
- Primary effusion lymphoma must be differentiated from other diseases such as:
- Plasmablastic lymphoma
- Burkitt lymphoma
- Pyothorax associated lymphoma
- Anaplastic large cell lymphoma
- Diffuse large B-cell lymphoma
Epidemiology and Demographics
Incidence
- The prevalence is unknown
- It accounts for less than 1% of non-AIDS related lymphomas and 3% of lymphomas associated with AIDS.
Age
- Primary effusion lymphoma is more commonly observed among young or middle aged patients.[3]
Gender
- Males are more commonly affected with primary effusion lymphoma than females.[3]
Risk Factors
- There are no established risk factors for primary effusion lymphoma.
Natural History, Complications and Prognosis
- Primary effusion lymphoma is a very fast-growing (aggressive) lymphoma
- They are 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.
- Prognosis is generally poor with average life expectancy of 3-4 months after diagnosis.
Diagnosis
Staging
Staging for primary effusion lymphoma is provided in the following table:[4]
| 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 |
Symptoms
- Symptoms of primary effusion lymphoma may include the following:[3]
- Fever
- Fatigue
- Weight loss
- Night sweats
- Painless swellings in the neck, axilla, groin, thorax, and abdomen
- Pain in the chest, abdomen, or bones
- Skin rash
Physical Examination
- Physical examination of primary effusion lymphoma may be remarkable for:
- Fever
- Skin rash
- Cervical lymphadenopathy
- Thoracic masses suggestive of central lymphadenopathy
- Abdominal masses suggestive of central lymphadenopathy
- Peripheral lymphadenopathy
Laboratory Findings
- There are no specific laboratory findings associated with primary effusion lymphoma.
- A lymph node biopsy is diagnostic of primary effusion lymphoma.
- 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.
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.
Other Diagnostic Studies
- Primary effusion lymphoma may also be diagnosed using bone marrow aspiraton and biopsy.
Treatment
Medical Therapy
- The mainstay of therapy for primary effusion lymphoma is chemotherapy and antiretroviral therapy.[1]
- Drug regimen: CHOP – Cyclophosphamide AND Doxorubicin AND Vincristine AND Prednisone
References
- ↑ 1.0 1.1 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
- ↑ Primary effusion lymphona. BioMed Central. http://jmedicalcasereports.biomedcentral.com/articles/10.1186/1752-1947-5-60. Accessed on March 23, 2016
- ↑ 3.0 3.1 3.2 Primary effusion lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf57e3e27c3994bd5378/. Accessed on March 23, 2016
- ↑ 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.