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

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

• The pathogenesis of [primary effusion lymphoma] is characterized by [feature1], [feature2], and [feature3].
• The [gene name] gene/Mutation in [gene name] has been associated with the development of [primary effusion lymphoma], involving the [molecular pathway] pathway.
• On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [primary effusion lymphoma].
• On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [primary effusion lymphoma].

Causes

• [Primary effusion lymphoma] may be caused by either [cause1], [cause2], or [cause3].
• [Primary effusion lymphoma] is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
• 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

• Common risk factors in the development of [primary effusion lymphoma] are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

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 [imaging study] findings associated with [primary effusion lymphoma].

• [Imaging study 1] is the imaging modality of choice for [primary effusion lymphoma].
• On [imaging study 1], [primary effusion lymphoma] is characterized by [finding 1], [finding 2], and [finding 3].
• [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

• [Primary effusion lymphoma] may also be diagnosed using [diagnostic study name].
• Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

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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