Primary effusion lymphoma

The primary effusion lymphoma subtype is also known as 'body cavity-based' lymphoma and is considered one of three types of AIDS-related lymphoma.

What is primary effusion lymphoma?

Primary effusion lymphoma (PEL) is a very rare sub-subtype of extranodal diffuse large B-cell lymphoma. It is more frequently found to be affecting young and middle-aged men, much more often than women, and only occasionally is it found in elderly patients.

Medically, the term 'effusion' means that some body fluid—typically blood but any body fluid will do—has escaped into a body cavity or surrounding tissue (thus the alias of body cavity-based lymphoma). 'Effusion' can also refer to that escaped fluid.

Thus, in PEL, lymphoma develops within the effusion. It does not typically cause a mass or tumor.

Symptoms and diagnosis of primary effusion lymphoma

Patients diagnosed with Primary effusion lymphoma are almost always found to be positive for the HIV virus. Other viruses often found in patients with PEL, specifically within the cancerous lymphocytes, include both Epstein-Barr virus (EBV) and human herpes virus 8, better known as Kaposi-sarcoma associated herpes virus. That said, there is a distinction between EBV-positive and EBV-negative PEL, although it is unclear whether this factors into progression, prognosis or treatment considerations.

PEL patients are found to have one or more of the following effusion-related conditions:

  • Pleural effusion (fluid that has escaped into the tissue that lines the lungs and chest cavity)
  • Pericardial effusion (escape of fluid into the pericardial cavity, which is the sac and tissue surrounding the heart)
  • Peritoneal effusion, better known as ascites (the escape of fluid into the cavity between the tissues lining the abdomen and its associated organs)

Prognosis of primary effusion lymphoma

Patients with primary effusion lymphoma have a poor prognosis, in large part because by the time of diagnosis not only is the lymphoma in advanced stage, the patients themselves are typically already highly immunocompromised. Death among these patients is generally caused by one of three diseases: the PEL, another cancer known as Kaposi's sarcoma, or an opportunistic infection characteristic of mortality in HIV/AIDS.

Researchers have noted that prognosis tends to be slightly better in (HIV positive) primary effusion lymphoma patients who are on antiretroviral therapy.

Sources

More Articles

More Articles

There are two types of cancer: benign and malignant. Benign cancers are the kind that don't spread and don't threaten one's life. Malignant...

Hodgkin's Disease—also referred to as Hodgkin's Lymphoma, these are the exact same diseases, just...

The Follicular Lymphoma International Prognostic Index, or FLIPI, is a standardized guide to help oncological diagnosticians accurately calculate...

Diffuse large B cell lymphoma prognosis is contingent upon several factors, and can be determined by using a well-...

The term 'metastatic lymphoma' does not refer to a diagnosis. Unlike many of the subtypes of lymphoma we have...

Diagnosing lymphoma is one of the more difficult diagnoses to make in cancer medicine, and contrary to perception, diagnosing lymphoma is not made...

Burkitt's lymphoma is an aggressive B-cell lymphoma that is common in children...

Treatment for Non-Hodgkin’s Lymphomas (NHL) differs from patient to patient. The regimen used depends on the type of...

Large Cell Lymphoma (LCL) is typically an aggressive (fast growing) cancer of either the B cell or T cell type. They are one of the most common...

Follicular lymphoma is classified as a Non-Hodgkin's Lymphoma. It is an indolent (slow-growing) cancer that affects...

Aggressive Non-Hodgkin’s Lymphomas (NHLs) are fast growing cancers (as opposed to indolent cancers). They involve...

Lymphoma is not difficult to diagnose once a patient and doctor begin to look for signs of cancer. However, Lymphoma–especially...

A lymphoma prognosis varies greatly depending on the type of lymphoma. There are more than 35 types of lymphoma, including 5 types of...

The lymphatic system, or lymph system, defends the body from foreign invasion by disease causing agents such as viruses, bacteria, or fungi. The...

Lymphoma is a cancer of the lymphatic system, causing B-cell or...