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Primary Effusion Lymphoma (PEL): A Rare and Aggressive Cancer

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Posted by fatima.shakeel@opmc.co | Jan 1, 2025

What is Primary Effusion Lymphoma (PEL)?

Primary Effusion Lymphoma (PEL) is a rare and aggressive form of B-cell lymphoma. It is strongly associated with human herpesvirus 8 (HHV8), also known as Kaposi Sarcoma-Associated Herpesvirus. This cancer typically develops in body cavities such as the chest, abdomen, or the lining of the heart.

Disease Epidemiology

PEL predominantly affects individuals with compromised immune systems, particularly those with low CD4 cell counts, a hallmark of HIV infection.
While it is most common in HIV-positive individuals, it can also occur in HIV-negative people, who often experience slightly better outcomes.

Causes

PEL is caused by HHV8 infection. Individuals at higher risk include those:

  • Living with HIV.
  • Undergoing immunosuppressive therapy after organ transplants.

Signs and Symptoms

Common signs and symptoms of PEL include:

  • Difficulty breathing or shortness of breath (dyspnea).
  • Persistent cough.
  • Chest discomfort.
  • Abdominal pain and swelling.
  • Swelling in joints.

Diagnosis

Diagnosis of PEL involves:

  1. Imaging Tests: Chest X-rays or CT scans to identify the tumour’s location, size, and spread.
  2. Laboratory Testing: Detection of HHV8 markers and B-cell-specific markers.

Treatment Options

1. Chemotherapy

  • DA-EPOCH: A regimen including etoposide.
  • CHOP: A combination of cyclophosphamide, doxorubicin, vincristine, and prednisone.
  • High-dose methotrexate may provide a slight survival benefit.
  • Antiviral medications are often used alongside chemotherapy.

2. Palliative Care

  • Talc Pleurodesis: A procedure to alleviate symptoms for patients who cannot tolerate intensive treatments.

3. Advanced Treatments

  • Immune Checkpoint Inhibitors: Pembrolizumab and similar therapies have shown promise in relapsed cases.
  • Stem Cell Therapy: Rarely used but may be an option in select cases.

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