Overview

Diffuse large B-cell lymphoma (DLBCL) is a type of blood cancer. Lymphomas are the most common type of blood cancers. There are two types of lymphoma: Hodgkin’s and non-Hodgkin’s. Diffuse large B-cell lymphoma is a non-Hodgkin’s lymphoma (NHL). Out of over 60 types of NHLs, diffuse large B-cell lymphoma is the most common. DLBCL is the most aggressive or fast-growing form of NHL. It can lead to death if left untreated.

All lymphomas, including DLBCL, affect the organs of your lymphatic system. The lymphatic system is what allows your body to fight infections. The organs that can be affected by lymphomas such as DLBCL include the following:

  • bone marrow
  • thymus
  • spleen
  • lymph nodes

The following features are what make a DLBCL different than other lymphomas:

  • It comes from abnormal B-cells.
  • These B-cells are larger than normal B-cells.
  • The abnormal B-cells are spread out instead of grouped together.
  • The abnormal B-cells will destroy the structure of the lymph node.

The main type of DLBCL is the most common of all DLBCL types. However, there are some less common types you may want to be aware of. These less common types of DLBCL are:

  • central nervous system lymphoma
  • T-cell/histiocyte-rich large B-cell lymphoma
  • EBV-positive DLBCL
  • primary mediastinal (thymic) large B-cell lymphoma
  • intravascular large B-cell lymphoma
  • ALK-positive large B-cell lymphoma

What are the symptoms?

The following are the primary symptoms you may experience with DLBCL:

  • enlarged lymph nodes
  • night sweats
  • unusual weight loss
  • loss of appetite
  • extreme tiredness or fatigue
  • fever
  • extreme itchiness

You may experience certain other symptoms depending on the location of your DLBCL. These additional symptoms may include:

  • abdominal pain, diarrhea, blood in stools
  • a cough and shortness of breath

What are the causes and risk factors?

A lymphoma occurs when lymphocytes begin to grow and divide, or reproduce, quickly and without control. The rapid growth of lymphocytes will cause them to interfere with other necessary functions of your immune system or your central nervous system. If the disease is left untreated, your body will not be able to fight infections.

The following are some possible risk factors for developing DLBCL:

  • Age. It usually affects those who are middle-aged or older, with 64 being the average age.
  • Ethnicity. It’s more likely to affect Caucasians.
  • Gender. Men have a slightly higher risk than women.

Family history does not affect your risk of developing DLBCL because it’s not an inherited disease.

Prognosis and survival rates

Two-thirds of people with DLBCL who are treated can be cured. However, if it’s left untreated, it can lead to death.

Most people with DLBCL are not diagnosed until the later stages. This is because you may not have outward symptoms until later. After diagnosis, your doctor will perform tests to determine the stage of your lymphoma. These tests may include some of the following:

Staging tells your medical team how far the tumors have spread throughout your lymphatic system. The stages for DLBCL are as follows:

  • Stage 1. Only one region or site is affected; this includes lymph nodes, lymph structure, or extranodal sites.
  • Stage 2. Two or more lymph node regions or two or more lymph node structures are involved. At this stage, the involved areas are on the same side of the body.
  • Stage 3. The involved lymph node regions and structures are on both sides of the body.
  • Stage 4. Other organs besides the lymph nodes and lymph structures are involved throughout your body. These organs may include your bone marrow, liver, or lungs.

These stages will also be accompanied by either an A or B after the stage number. The letter A means you are not having the common symptoms of fever, night sweats, or weight loss. The letter B means that you are having these symptoms.

In addition to the staging and the A or B status, your doctor will also give you an IPI score. IPI stands for International Prognostic Index. The IPI score ranges from 1 to 5 and is based on how many of factors you have that may lower your survival rate. These five factors are:

  • being over 60 years of age
  • having a higher than normal level of lactate dehydrogenase, a protein found in your blood
  • being in poor overall health
  • having the disease in stage 3 or 4
  • involvement of more than one extranodal disease site

All three of these diagnostic criteria will be combined to give you a prognosis. They will also help your doctor determine the best treatment options for you.

How is it treated?

Treatment of DLBCL is determined by several factors. However, the most important factor your doctor will use to determine treatment options is whether your disease is localized or advanced. Localized means it hasn’t spread. Advanced is usually when the disease has spread to more than one location in your body.

The treatments commonly used on DLBCL are chemotherapy drugs, radiation treatments, or immunotherapy. Your doctor may also prescribe a combination of the three treatments. The most common chemotherapy treatment is referred to as R-CHOP. R-CHOP stands for a combination of the chemotherapy and immunotherapy medications rituximab, cyclophosphamide, doxorubicin, and vincristine, along with prednisone. R-CHOP is given through an IV for four of the medications, and prednisone is taken by mouth. R-CHOP is usually administered every three weeks.

Chemotherapy drugs work by slowing down the ability of the rapidly growing cancer cells to reproduce. Immunotherapy drugs target groups of cancer cells with antibodies and work to destroy them. The immunotherapy drug, rituximab, specifically targets the B-cells or lymphocytes. Rituximab can affect the heart and may not be an option if you have certain heart conditions.

Treatment for localized DLBCL will usually include approximately three rounds of R-CHOP along with radiation therapy. Radiation therapy is a treatment where high-intensity X-rays are aimed at the tumors.

Treatment for advanced DLBCL

Advanced DLBCL is treated with the same R-CHOP combination of chemotherapy and immunotherapy medications. However, advanced DLBCL requires more rounds of the medications that are administered every three weeks. Advanced DLBCL will usually need six to eight rounds of the treatment. Your doctor will usually take another PET scan at the midway point of treatment to make sure it’s working effectively. Your doctor may include additional rounds of treatment if the disease is still active or it returns.

Young adults or children with DLBCL have a higher rate of reoccurrence. For this reason, your doctor may recommend a stem cell transplant to help avoid a reoccurrence. This treatment is done after you’re treated with the R-CHOP regimen.

How is it diagnosed?

DLBCL is diagnosed by removing part or all of the lump, swollen lymph node, or area with the abnormalities and doing a biopsy on the tissue. Depending on various factors, including the location of the affected area, this procedure may be done under general or local anesthesia.

Your doctor will also interview you for details on your medical issues and symptoms and give you a physical exam. After confirmation from the biopsy, your doctor will perform some additional tests to determine the stage of your DLBCL.

Outlook

DLBCL is considered a curable disease when treated as early as possible. The sooner you are diagnosed, the better your outlook will be. The treatments for DLBCL can have serious side effects. Be sure to discuss these before starting your treatment.

Despite the side effects, it’s important that you treat your DLBCL quickly and as early as possible. Seeing your doctor at the first signs of symptoms and getting treated is crucial. If it’s left untreated, it can be life-threatening.