Combination Therapy for Primary Mediastinal B-Cell Lymphoma

Primary mediastinal B-cell lymphoma (PMBL) is a fast-growing type of non-Hodgkin's lymphoma. This cancer can spread quickly through your chest and make it hard to breathe. It can also cause other symptoms. Getting the right treatment as early as possible is important.

Treatment includes a group of chemotherapy drugs, plus rituximab (Rituxan). It could also include radiation. Yet the exact combination of drugs doctors use has stirred some debate.

Very few studies compare the different combination treatments. So doctors don't know for sure which works best. But they do know that combining drugs offers a good chance for a cure for this cancer.

Before you start your treatment, your doctor will explain which medicines you'll get and why they're the best choices for you.

R-CHOP

The main treatment for PMBL is with a drug combination known as R-CHOP. It contains:

  • C = Cyclophosphamide
  • H = Doxorubicin hydrochloride (Hydroxydaunorubicin)
  • O = Vincristine sulfate (Oncovin)
  • P = Prednisone

The first three -- C, H, and O -- are chemotherapy drugs. Prednisone is a corticosteroid.

Each of these medicines attacks the cancer differently. Experts believe the four drugs work better together than each drug does alone.

The "R" in the treatment name stands for rituximab. It's a type of immunotherapy called a monoclonal antibody. Rituximab targets a protein called CD20 that sits on PMBL cells. When the medicine binds to this protein, the cancer cell dies. Adding rituximab to chemotherapy may make things better for people with this type of cancer.

Both the chemotherapy drugs and Rituxan are given through a vein (IV). You get chemotherapy in cycles. Each cycle lasts for about 3 weeks. You get three to six cycles.

Many people who get R-CHOP for PMBL will also get radiation to their chest afterward to make sure their cancer is cured.

Your doctor may do a positron emission tomography (PET) scan after you're done with your chemotherapy. This is done to see if you have any cancer left in your chest. If you don't, your doctor will watch you, but you probably won't need more treatment.

If the PET scan shows that you still have lymphoma cells in your chest, you may need radiation.

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EPOCH-R

This includes a group of five drugs:

  • E = Etoposide phosphate
  • P = Prednisone
  • O = Vincristine sulfate (Oncovin)
  • C = Cyclophosphamide
  • H = Doxorubicin hydrochloride (Hydroxydaunorubicin)

Rituxan is added to the five other drugs.

You'll get EPOCH-R in a hospital six times, once every 3 weeks. Each infusion takes 4 days. Your doctor will do blood tests between treatments and tweak your chemotherapy doses as needed.

The advantage of EPOCH-R over R-CHOP is that it takes little or no radiation. Radiation treatment can raise your chance of cancer or heart disease in the future.

Choosing a Treatment

Most people with PMBL are cured.

Which treatment you get may depend on the cancer center you visit, and your risks. R-CHOP along with radiation could be a better choice if there's a low risk of your cancer coming back. EPOCH-R might be the choice for you if your cancer is hard to treat and there's a chance it could come back.

When at least 2 previous treatments have failed, a treatment called CAR (chimeric antigen receptor) T-cell therapy is sometimes used in adults. It's a type of gene therapy. 

Your doctor will consider the benefits and side effects of each treatment to find the best option for you.

WebMD Medical Reference Reviewed by Laura J. Martin, MD on May 06, 2018

Sources

SOURCES:

American Cancer Society: "Treating B-Cell Non-Hodgkin Lymphoma."

Blood Journal: "Primary mediastinal B-cell lymphoma and mediastinal gray zone lymphoma: Do they require a unique therapeutic approach?"

BMC Cancer: "Addition to rituximab to CHOP-like chemotherapy in first line treatment of primary mediastinal B-cell lymphoma," "The treatment of primary mediastinal large B-cell lymphoma: A two decades monocentric experience with 98 patients."

Cancer Network: "Management of primary mediastinal B-cell lymphoma and gray zone lymphoma," "Treatment strategies in primary mediastinal B-cell lymphoma."

Dana-Farber Cancer Institute: "Primary Mediastinal B-Cell Lymphoma."

Macmillan Cancer Support: "R-CHOP Chemotherapy."

National Cancer Institute: "CHOP," "R-EPOCH."

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