Primary effusion lymphoma

To view the landing page of lymphoma, click here.

WikiDoc Resources for Primary effusion lymphoma
Articles
Most recent articles on Primary effusion lymphoma Most cited articles on Primary effusion lymphoma Review articles on Primary effusion lymphoma Articles on Primary effusion lymphoma in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Primary effusion lymphoma Images of Primary effusion lymphoma Photos of Primary effusion lymphoma Podcasts & MP3s on Primary effusion lymphoma Videos on Primary effusion lymphoma
Evidence Based Medicine
Cochrane Collaboration on Primary effusion lymphoma Bandolier on Primary effusion lymphoma TRIP on Primary effusion lymphoma
Clinical Trials
Ongoing Trials on Primary effusion lymphoma at Clinical Trials.gov Trial results on Primary effusion lymphoma Clinical Trials on Primary effusion lymphoma at Google
Guidelines / Policies / Govt
US National Guidelines Clearinghouse on Primary effusion lymphoma NICE Guidance on Primary effusion lymphoma NHS PRODIGY Guidance FDA on Primary effusion lymphoma CDC on Primary effusion lymphoma
Books
Books on Primary effusion lymphoma
News
Primary effusion lymphoma in the news Be alerted to news on Primary effusion lymphoma News trends on Primary effusion lymphoma
Commentary
Blogs on Primary effusion lymphoma
Definitions
Definitions of Primary effusion lymphoma
Patient Resources / Community
Patient resources on Primary effusion lymphoma Discussion groups on Primary effusion lymphoma Patient Handouts on Primary effusion lymphoma Directions to Hospitals Treating Primary effusion lymphoma Risk calculators and risk factors for Primary effusion lymphoma
Healthcare Provider Resources
Symptoms of Primary effusion lymphoma Causes & Risk Factors for Primary effusion lymphoma Diagnostic studies for Primary effusion lymphoma Treatment of Primary effusion lymphoma
Continuing Medical Education (CME)
CME Programs on Primary effusion lymphoma
International
Primary effusion lymphoma en Espanol Primary effusion lymphoma en Francais
Business
Primary effusion lymphoma in the Marketplace Patents on Primary effusion lymphoma
Experimental / Informatics
List of terms related to Primary effusion lymphoma

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]

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

[Disease name] was first discovered by [name of scientist], a [nationality + occupation], in [year]/during/following [event].

The association between [important risk factor/cause] and [disease name] was made in/during [year/event].

In [year], [scientist] was the first to discover the association between [risk factor] and the development of [disease name].

In [year], [gene] mutations were first implicated in the pathogenesis of [disease name].

There have been several outbreaks of [disease name], including -----.

In [year], [diagnostic test/therapy] was developed by [scientist] to treat/diagnose [disease name].

Classification

There is no established system for the classification of [disease name].

OR

[Disease name] may be classified according to [classification method] into [number] subtypes/groups: [group1], [group2], [group3], and [group4].

OR

[Disease name] may be classified into [large number > 6] subtypes based on [classification method 1], [classification method 2], and [classification method 3]. [Disease name] may be classified into several subtypes based on [classification method 1], [classification method 2], and [classification method 3].

OR

Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.

OR

If the staging system involves specific and characteristic findings and features: According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].

OR

The staging of [malignancy name] is based on the [staging system].

OR

There is no established system for the staging of [malignancy name].

Pathophysiology

• Primary effusion lymphoma is associated with human herpes virus 8 (HHV8) infection and Epstein-Barr virus (EBV) infection.
• Among a very few of patients with human herpes virus 8 (HHV8) not associated with an effusion (a solid variant of PEL).
• 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.

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 primary effusion lymphoma (PEL) cases.

Differentiating primary effusion lymphoma from other Diseases

• Primary effusion lymphoma must be differentiated from other diseases such as:

• 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.
• Primary effusion lymphoma usually has same age, race of HIV patients.

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.

Age

• Primary effusion lymphoma is more commonly observed among young or middle aged patients.

Gender

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

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:

• • 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.
• They are usually confined to pleural, pericardial, peritoneal cavities, presenting as serous effusions without detectable tumor masses.
• Prognosis is generally poor with average life expectancy of 3-4 months after diagnosis.

Diagnosis

• Among the patients who present with clinical signs of PEL usually have HIV infection or Kaposi's sarcoma or Castleman's disease.
• Primary effusion lymphoma diagnosed by pathological analysis of involved tissue .
• 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.

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:

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:

• 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
• 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.

• 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.

Electrocardiogram

There are no ECG findings associated with Primary effusion lymphoma .

X-ray

There are no x-ray findings associated with [disease name].

OR

An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

Echocardiography or Ultrasound

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

OR

Echocardiography/ultrasound may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no echocardiography/ultrasound findings associated with [disease name]. However, an echocardiography/ultrasound may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

CT scan

There are no CT scan findings associated with [disease name].

OR

[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

MRI

There are no MRI findings associated with [disease name].

OR

[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].

OR

There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].

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.

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.
• Drug regimen: CHOP – Cyclophosphamide AND Doxorubicin AND Vincristine AND Prednisone

Surgery

Surgical intervention is not recommended for the management of [disease name].

OR

Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3]

OR

The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].

OR

The feasibility of surgery depends on the stage of [malignancy] at diagnosis.

OR

Surgery is the mainstay of treatment for [disease or malignancy].

Primary Prevention

There are no established measures for the primary prevention of [disease name].

OR

There are no available vaccines against [disease name].

OR

Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].

OR

[Vaccine name] vaccine is recommended for [patient population] to prevent [disease name]. Other primary prevention strategies include [strategy 1], [strategy 2], and [strategy 3].

Secondary Prevention

There are no established measures for the secondary prevention of [disease name].

OR

Effective measures for the secondary prevention of [disease name] include [strategy 1], [strategy 2], and [strategy 3].