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

In T cell lymphoma, T lymphocytes, which are an essential part of the body's immune response, become malignant. T cell lymphomas account for about...

Lymphomatoid Papulosis (LyP) is a rare skin disorder that involves cancerous looking skin lesions. It is more than a skin condition; it is the...

In non-Hodgkin's lymphoma, you have your B-cell lymphomas and you have your T-cell lymphomas.

Why B...

MALT lymphoma is a rare B-cell non-Hodgkin's lymphoma that typically runs an indolent or slow-growing clinical...

Mantle cell lymphoma (MCL) is a relatively rare B-cell subtype of non-Hodgkin'...

T-Cell Lymphoblastic Lymphoma (T-LBL) is a very rare subtype of non-Hodgkin's lymphoma. It tends to develop in...

In general, a diagnosis of T-cell lymphoma denotes a poorer prognosis than a diagnosis of B-cell lymphoma. One of...

Mantle cell lymphoma (MCL) is just one of 50-60 known B-cell subtypes of...

After some of the dust has settled, the thoughts of many new lymphoma patients turn to diet and nutrition. They want to know if, in the past,...

The term NK T cell lymphoma refers to one of two subtypes of lymphoma that affect the NK (Natural Killer)...

In a perfect world, every case of cancer would respond to, and be cured by first-line therapy. Unfortunately, it is not often the case. This is...

Prednisone is a glucocorticosteroid (a steroid) used in the treatment of many types of cancers. It functions as an anti-inflammatory medicine that...

In 1964, researchers at the National Cancer Institute developed the first combination chemotherapy that cured a...