Primary effusion lymphoma: Current concepts and management

Nivedita Arora, Arjun Gupta, Navid Sadeghi

Research output: Contribution to journalReview article

11 Citations (Scopus)

Abstract

Purpose of review To summarize the current epidemiology, management, and outcomes of primary effusion lymphoma (PEL) and highlight possible future research efforts. Recent findings Cyclophosphamide, doxorubicin, vincristine, prednisone-based chemotherapy regimens alone or in combination with immunomodulatory agents (e.g., lenalidomide), or proteasome inhibitors (e.g., bortezomib), or targeted therapies, are commonly used to treat PEL. Highly active antiretroviral therapy should be continued or initiated in patients with HIV infection. Randomized controlled trials are lacking. Prognosis remains grim and there exists a need for further investigation into optimal treatment strategies. Summary PEL is an aggressive mature B-cell neoplasm primarily seen in young to middle aged men with HIV, though immunosuppression related to age and comorbidities such as cirrhosis or organ transplantation also predisposes to PEL. Classic cavitary PEL presents as an effusion in the pleural, pericardial, or peritoneal space. Human herpes virus-8/Kaposi's sarcoma herpes virus) is classically detected. Given its rarity, randomized controlled trials evaluating optimal treatment regimens are lacking, and cyclophosphamide, doxorubicin, vincristine, prednisone-based chemotherapy has been the mainstay of treatment. Advancement in knowledge of the oncogenic signaling pathways involved in Kaposi's sarcoma herpes virus-induced tumorigenesis may pave the way to develop targeted therapies. Video abstract http://links.lww.com/COPM/A19

Original languageEnglish (US)
Pages (from-to)365-370
Number of pages6
JournalCurrent Opinion in Pulmonary Medicine
Volume23
Issue number4
DOIs
StatePublished - Jul 1 2017

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Primary Effusion Lymphoma
Kaposi's Sarcoma
Vincristine
Prednisone
Viruses
Doxorubicin
Cyclophosphamide
Randomized Controlled Trials
Therapeutics
Drug Therapy
Proteasome Inhibitors
Highly Active Antiretroviral Therapy
Organ Transplantation
Pleural Effusion
Immunosuppression
HIV Infections
Comorbidity
Carcinogenesis
Epidemiology
Fibrosis

Keywords

  • HIV
  • human herpes virus-8
  • Kaposi's sarcoma herpes virus
  • management
  • outcomes
  • primary effusion lymphoma

ASJC Scopus subject areas

  • Medicine(all)
  • Pulmonary and Respiratory Medicine

Cite this

Primary effusion lymphoma : Current concepts and management. / Arora, Nivedita; Gupta, Arjun; Sadeghi, Navid.

In: Current Opinion in Pulmonary Medicine, Vol. 23, No. 4, 01.07.2017, p. 365-370.

Research output: Contribution to journalReview article

Arora, Nivedita ; Gupta, Arjun ; Sadeghi, Navid. / Primary effusion lymphoma : Current concepts and management. In: Current Opinion in Pulmonary Medicine. 2017 ; Vol. 23, No. 4. pp. 365-370.
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