B-cell prolymphocytic leukemia (B-PLL) is a rare, aggressive type of leukemia that progresses rapidly. People with B-PLL typically have a less favorable outlook. However, new targeted treatments in development may improve outcomes.

This cancer affects immature cells in the immune system called prolymphocytes, which usually develop into white blood cells called lymphocytes. In B-PLL, B-lymphocytes grow and divide excessively, crowding out healthy cells.

B-PLL has a high rate of recurrence, and the outlook for people with this cancer is generally less favorable. However, new treatment options are emerging through ongoing research.

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B-PLL is rare and accounts for fewer than 1% of all leukemia cases affecting lymphocytes.

In B-PLL, immature forms of B-lymphocytes, called prolymphocytes, divide and multiply rapidly in the bone marrow and blood.

B-lymphocytes are white blood cells that produce antibodies to fight infections and viruses. However, in B-PLL, the cell growth is out of control, and the lymphocyte count is excessive. The B-lymphocytes eventually crowd out other healthy cells, such as red blood cells and platelets.

The condition typically progresses rapidly and aggressively.

What is leukemia?

Leukemia is cancer of the blood cells. It usually begins in the bone marrow, where blood cells form. These cells include:

  • white blood cells, which help fight infection
  • red blood cells, which deliver oxygen from the lungs to the organs and bodily tissues
  • platelets, which form blood clots to stop bleeding

When someone has leukemia, their bone marrow produces excessive atypical blood cells. The cancer most often affects the white blood cells, which build up in the bone marrow and crowd out other healthy blood cells.

Learn more about leukemia.

In B-PLL, more than 55% of the B-lymphocytes in the blood are prolymphocytes.

B-PLL may also cause:

A person should contact a doctor if they have any symptoms or early signs of leukemia.

Early signs of leukemia may resemble flu-like symptoms that do not resolve. They can include:

A person may also experience:

Researchers do not fully understand the causes of B-PLL.

Some believe it may develop as a progression of slow-growing B-cell cancers, such as mantle cell lymphoma (MCL) or chronic lymphocytic leukemia (CLL). However, some researchers debate this idea.

Genetics may also play a role in B-PLL. Although further studies are necessary, some research suggests abnormalities in the MYC and TP53 genes may have a link with the condition.

Certain groups have a higher risk of B-PLL than the larger population. These include:

  • males
  • older adults, as the median age of diagnosis is 69 years
  • people with a family history of leukemia

Risk factors that may increase a person’s chances of developing leukemia in general include:Trusted Source

There is no standard treatment for B-PLL. Doctors may recommend different treatments depending on various factors, including a person’s age and overall health.

Treatment can include:

  • Monitoring: If a person has no symptoms, doctors may actively monitor their health and wait to begin treatment. This can involve frequent blood tests.
  • Chemotherapy and immunotherapy: Doctors may treat B-PLL with a combination of chemotherapy drugs, including bendamustine (Bendeka), rituximab (Rituxan), fludarabine (Fludara), and cyclophosphamide (Cytoxan). However, B-PLL is usually challenging to manage with chemotherapy and may not respond to the treatment.
  • Hematopoietic stem cell transplant: This treatment involves a doctor transplanting blood, bone marrow, or umbilical cord stem cells from a donor or the person’s own body. This is not suitable for everyone with B-PLL. However, a doctor may recommend it for people in good general health who have responded to initial therapies.
  • Targeted therapies: Doctors may use the monoclonal antibody alemtuzumab (Campath) to treat B-PLL in people with TP53 gene mutations whose cancer has been resistant to other treatments. Other targeted therapies include ibrutinib (Imbruvica) and idelalisib (Zydelig), although the Food and Drug Administration (FDA) has not yet approved these to treat B-PLL.

The outlook for people with B-PLL is typically less favorable. The cancer often progresses rapidly and may be resistant to standard chemotherapy and immunotherapy treatments. A person may require aggressive drug therapy to treat B-PLL.

The median survival time with B-PLL is around 3 years.

Several factors may affect a person’s outlook. These include:

  • being older than 70 years
  • having the following:
    • high risk genetic mutations, such as the TP53 deletion
    • other health problems
    • a history of leukemia

However, new treatments in current research show promising results and may improve a person’s outlook.

As researchers do not know the precise causes of B-PLL, a person cannot prevent the cancer. Certain potential risk factors for B-PLL, such as age and genetics, cannot change.

A person may help reduce their risk of developing leukemia by making certain lifestyle choices, such as:

  • not smoking
  • maintaining a moderate weight for their body type
  • avoiding exposure to benzene and formaldehyde

Support is available for people with leukemia and their loved ones. Support groups, charities, and other organizations provide various helpful resources to help people understand and manage their condition.

Organizations that provide leukemia support include:

B-cell prolymphocytic leukemia (B-PLL) is a type of leukemia that progresses rapidly. People with B-PLL typically have a less favorable outlook. However, new treatments in the research process, including hematopoietic stem cell transplants and targeted therapies, show promising results.