Primary effusion lymphoma

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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

Overview

Primary effusion lymphoma (PEL) is rare subtype of diffuse large B-cell lymphoma (DLBCL).

Historical Perspective

• [Primary effusion lymphoma] was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
• In [year], [gene] mutations were first identified in the pathogenesis of [primary effusion lymphoma].
• In [year], the first [discovery] was developed by [scientist] to treat/diagnose [primary effusion lymphoma].

Classification

• [Primary effusion lymphoma] may be classified according to [classification method] into [number] subtypes/groups:

• [group1]
• [group2]
• [group3]

• Other variants of [primary effusion lymphoma] include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

• Primary effusion lymphoma is associated with human herpes virus 8 (HHV8) infection and Epstein-Barr virus (EBV) infection.
• Primary effusion lymphoma most often occurs in people with weakened immune systems, 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.

• 

  At light microscopy, the sample consisted of a frankly neoplastic population provided with plasmablastic and/or anaplastic morphology (Figure 1A), which turned out CD3-, CD20- (Figure 1B), CD79a-, CD45+ (Figure 1C), CD38+, CD30+, IRF4+, LANA-1+ (Figure 1D), EBER+ (Figure 1C inset), and Ki-67>90%. Based on these findings, we made a diagnosis of Primary effusion lymphoma.

Causes

• There are no established causes for primary effusion lymphoma.

Differentiating [primary effusion lymphoma] from other Diseases

• [Primary effusion lymphoma] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:

• [Differential dx1]
• [Differential dx2]
• [Differential dx3]

Epidemiology and Demographics

• The prevalence of [primary effusion lymphoma] is approximately [number or range] per 100,000 individuals worldwide.
• In [year], the incidence of [primary effusion lymphoma] was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

• Patients of all age groups may develop [primary effusion lymphoma].

• [Primary effusion lymphoma] is more commonly observed among patients aged [age range] years old.
• [Primary effusion lymphoma] is more commonly observed among [elderly patients/young patients/children].

Gender

• [Primary effusion lymphoma] affects men and women equally.

• [Gender 1] are more commonly affected with [primary effusion lymphoma] than [gender 2].
• The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

• There is no racial predilection for [primary effusion lymphoma].

• [Primary effusion lymphoma] usually affects individuals of the [race 1] race.
• [Race 2] individuals are less likely to develop [primary effusion lymphoma].

Risk Factors

• There are no established risk factors for primary effusion lymphoma.

Natural History, Complications and Prognosis

• The majority of patients with [primary effusion lymphoma] remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• If left untreated, [#%] of patients with [primary effusion lymphoma] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Common complications of [primary effusion lymphoma] include [complication 1], [complication 2], and [complication 3].
• Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with [primary effusion lymphoma] is approximately [#%].

Diagnosis

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

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
• 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

• There is no treatment for [primary effusion lymphoma]; the mainstay of therapy is supportive care.

• The mainstay of therapy for [primary effusion lymphoma] is [medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

• Surgery is the mainstay of therapy for [primary effusion lymphoma].
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of [primary effusion lymphoma].
• [Surgical procedure] can only be performed for patients with [disease stage] [primary effusion lymphoma].

Prevention

• There are no primary preventive measures available for [primary effusion lymphoma].

• Effective measures for the primary prevention of [primary effusion lymphoma] include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with [primary effusion lymphoma] are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].

References

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