Primary effusion lymphoma: Difference between revisions

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=== Symptoms ===
=== Symptoms ===
*Symptoms of primary effusion lymphoma may include the following::<ref name=seer.cancer.gov>Primary effusion lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf57e3e27c3994bd5378/. Accessed on March 22, 2016 </ref>
*Symptoms of primary effusion lymphoma may include the following:<ref name=seer.cancer.gov>Primary effusion lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf57e3e27c3994bd5378/. Accessed on March 22, 2016 </ref>
:* [[Fever]]
:* [[Fever]]
:* [[Fatigue]]
:* [[Fatigue]]

Revision as of 19:33, 23 March 2016

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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.[1]
  • 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.[2]

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 such as:

Epidemiology and Demographics

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 females.

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:[3]

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:[4]

Physical Examination

  • Physical examination of primary effusion lymphoma may be remarkable for:

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

  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
  2. 2.0 2.1 Primary effusion lymphona. BioMed Central. http://jmedicalcasereports.biomedcentral.com/articles/10.1186/1752-1947-5-60. Accessed on March 23, 2016
  3. 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.
  4. Primary effusion lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf57e3e27c3994bd5378/. Accessed on March 22, 2016


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