Lymphoplasmacytic lymphoma (LPL) is a type of non-Hodgkin’s lymphoma that commonly causes anemia or swollen lymph nodes.
Lymphoplasmacytic lymphoma (LPL) is a rare type of slowly developing cancer that mostly affects older adults.
Lymphomas are cancers of the lymph system, a part of your immune system that helps fight off infections. In lymphoma, white blood cells called B lymphocytes or T lymphocytes grow out of control because of a mutation.
In LPL, abnormal B lymphocytes reproduce in your bone marrow and displace existing healthy blood cells. This also leads to lower production of new healthy blood cells.
B lymphocytes, also known as B cells, typically move from your bone marrow to your spleen and lymph nodes. There, they may become plasma cells, producing antibodies to combat infections.
If you don’t have enough healthy blood cells, it compromises your immune system. This may result in:
- anemia, a shortage of red blood cells
- neutropenia, a shortage of a type of white blood cell called neutrophils, which increases infection risk
- thrombocytopenia, a shortage of blood platelets, which increases bleeding and bruising risks
The most common type of LPL is Waldenstrom macroglobulinemia (WM), which is characterized by abnormal production of immunoglobulin (antibodies). WM is sometimes mistakenly referred to as identical to LPL, but it’s actually a subset of LPL.
LPL is slow-growing cancer, and about
Other symptoms of LPL may include:
- weakness and fatigue (often caused by anemia)
- fever, night sweats, and weight loss (generally associated with B-cell lymphomas)
- blurred vision
- nose bleeds
- bleeding gums
- elevated beta-2-microglobulin, a blood marker for tumors
The cause of LPL isn’t fully understood. Researchers are investigating several possibilities:
- There may be a genetic component, as about
20%of people with WM have a first-degree relative with LPL or a similar non-Hodgkin lymphoma (NHL). Some studieshave found that lymphomas like LPL may be associated with autoimmune diseases like Sjögren syndrome or with the hepatitis C virus, but other studies haven’t shown this link.
- People with LPL
commonly havecertain genetic mutations that aren’t inherited.
The diagnosis of LPL is difficult and usually made after excluding other possibilities. An increase in immunoglobulin is not enough to begin a diagnosis process and initiate treatment if needed. A person usually needs to have noticeable symptoms.
LPL can resemble other B-cell lymphomas with similar types of plasma cell differentiation. These include:
- mantle cell lymphoma
- chronic lymphocytic leukemia
- marginal zone lymphoma
- plasma cell myeloma
Your doctor will examine you physically and ask for your medical history. They’ll order blood work and possibly a bone marrow or lymph node biopsy to look at the cells under a microscope.
Your doctor may also use other tests to rule out similar cancers and determine the stage of your disease. These may include:
- chest X-ray
- CT scan
- PET scan
There are multiple treatments for lymphoplasmacytic lymphoma:
- watch and wait
- biological therapy
- targeted therapy
- stem cell transplants
- clinical trials
Watch and wait
LBL is slow-growing cancer. You and your doctor may decide to wait and monitor your blood regularly before starting treatment.
According to the
Several drugs that work in different ways, or combinations of drugs, may be used to kill cancer cells. These include:
- bendamustine (Belrapo, Bendeka, Treanda)
- chlorambucil (Leukeran)
- cyclophosphamide (Cytoxan)
- bendamustine (Belrapo, Bendeka, Treanda)
- a combination of thalidomide (Thalomid) and rituximab
- a combination of cyclophosphamide, dexamethasone, and rituximab (Rituxan)
- a combination of bortezomib (Velcade), dexamethasone (in some cases), and rituximab (Rituxan)
- a combination of cyclophosphamide, prednisone, rituximab (Rituxan) (in some cases), and vincristine
The particular regimen of drugs will vary depending on your general health, your symptoms, and possible future treatments.
Biological therapy drugs are human-made substances acting like your immune system to kill the lymphoma cells. These drugs
Some of these human-made antibodies, called monoclonal antibodies, are:
- rituximab (Rituxan)
- ofatumumab (Arzerra)
Other biological drugs are immunomodulating drugs (IMiDs) and cytokines.
Targeted therapy drugs aim to block particular cell changes that cause cancer.
Some of these drugs have been used to combat other cancers and are now being researched for LBL. In general, these drugs block proteins that allow the lymphoma cells to keep growing. Examples include:
- bortezomib (Velcade)
- carfilzomib (Kyprolis)
- everolimus (Afinitor)
Researchers are now studying additional medications such as temsirolimus (Torisel).
Stem cell transplants
In this treatment, blood-forming stem cells are removed from a donor’s bloodstream and stored frozen. Then a high dose of chemotherapy or radiation is used to kill all the bone marrow cells (healthy and cancerous) in the person with LBL. The preserved blood-forming cells are then put into the bloodstream.
The stem cells may come from the person being treated (autologous) or from someone close to the person (allogenic).
Be aware that stem cell transplants are still in the experimental stage. Also, there are short-term and long-term side effects from these transplants.
As with many kinds of cancer, new therapies are under development, and you may find a clinical trial to participate in. Ask your doctor about this and visit ClinicalTrials.gov for more information.
Here are answers to some additional questions on lymphomas and LPL.
Is lymphoplasmacytic lymphoma curable?
As of now, LPL has no cure. Your LPL may go into remission but later reappear. Treatment is usually focused on managing the symptoms.
What’s the difference between lymphoplasmacytic lymphoma and other lymphomas?
Lymphomas are broadly categorized as non-Hodgkin’s lymphomas or Hodgkin’s lymphomas.
Hodgkin’s lymphomas have a specific kind of abnormal cell called the Reed-Sternberg cell.
Non-Hodgkin’s lymphomas are distinguished by where the cancers start and the specific characteristics of the malignant cells.
LPL is a non-Hodgkin’s lymphoma. It’s a very rare lymphoma, making up only about
What is the life expectancy with lymphoma?
According to the ACS, the relative 5-year survival rate for people with non-Hodgkin’s lymphoma is
Who gets lymphoplasmacytic lymphoma?
There are about
LPL is slow-growing cancer that causes abnormal B lymphocytes to reproduce in the bone marrow. This makes it hard for healthy blood cells to do their job and for new healthy blood cells to form. Ultimately, LPL can compromise your immune system, making you more susceptible to illnesses.
However, LPL is very treatable. The median survival of LPL patients is about 5 years, and about
If your symptoms are properly managed, you have a greater chance of living longer.