Lymphocyte-rich Hodgkin lymphoma (LRHL) is a less common type of Hodgkin lymphoma. (Hodgkin lymphoma is the type of cancer that occurs when certain white blood cells in the immune system — known as lymphocytes — divide and multiply out of control.) Typically, Hodgkin lymphoma (also known as Hodgkin’s disease) occurs mostly in a person’s lymphatic system. However, Hodgkin lymphoma cancerous cells can also spread to other places in the body, such as the bone marrow.

There are two main types of Hodgkin lymphoma: classical Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma. Classical Hodgkin lymphoma, which includes LRHL, occurs more often. In fact, the Leukemia & Lymphoma Society reports classical Hodgkin lymphoma accounts for 95 percent of the cases of all Hodgkin lymphoma.

Within classical Hodgkin lymphoma, subtypes (and their frequencies) include:

• Nodular sclerosis (NSHL) — 70 percent
• Mixed cellularity (MCCHL) — 20 percent to 25 percent
• LRHL — 5 percent
• Lymphocyte-depleted (LDHL) — 1 percent to 1.5 percent

There are some unique features of LRHL that make its early signs and symptoms different from what is observed with other types of classical Hodgkin lymphoma. It also shares some features with other types of non-Hodgkin lymphoma.

Early Signs and Symptoms of LRHL

The early signs and symptoms of LRHL differ in some ways from the symptoms observed with other forms of classical Hodgkin lymphoma. The B symptoms typically experienced by most people with other types of classical Hodgkin lymphoma include night sweats, fever, and weight loss. However, only about 10 percent of people with LRHL will experience B symptoms.

Because B symptoms rarely show up in people with LRHL, it’s possible that few changes in the body will indicate a person has the disease. Some of the early symptoms that do appear in people with LRHL include fatigue and swollen or enlarged lymph nodes. These swollen lymph nodes are usually in the neck or armpits. Large tumor masses are not common in people with LRHL.

Even if you don’t have any masses, a doctor usually still needs to perform a biopsy and examine tissue samples for signs of LRHL — or another type of cancer. If they suspect LRHL, they will send the samples to a pathologist who then looks specifically for Reed-Sternberg cells. (Reed-Sternberg cells are cancerous cells that come from a type of lymphocyte called a B cell.) In someone with LRHL, Reed-Sternberg cells can appear in a pattern that is more scattered than in other classical Hodgkin lymphomas. Also, someone with LRHL has many normal lymphocytes, too. If the pathologist finds these two things — a scattered pattern and normal lymphocytes along with those that are cancerous — your doctor can confirm a diagnosis of LRHL.

Other Differentiators of LRHL

There are some other defining characteristics that make LRHL unique from the other types of classical Hodgkin lymphoma. For example, the average age of a person newly diagnosed with LRHL is older — 43 years old — when compared to people with other types of classical Hodgkin lymphomas. Even so, most lymphocyte-rich Hodgkin lymphomas are caught in the early stages (stages 1 and 2) of the disease. That’s in part because they might have swollen lymph nodes in areas (like the neck) that can easily be felt in a physical exam. People with LRHL have a better prognosis (outlook) than people with other forms of classical Hodgkin lymphoma. The complete remission rate is typically 95 percent, while the relapse rate (when cancer comes back after treatment) is 17 percent.

What Causes LRHL?

The cause of LRHL is thought to be similar to some of the other forms of classical Hodgkin lymphoma — genetic mutations, as well as infection with Epstein-Barr virus (EBV).

Genetic mutations can increase cell survival and activity (cause a cancer cell to live longer and grow more). Those traits give abnormal cells an advantage over normal cells and allow them to replicate out of control. Further, B cells can also become infected with EBV. That factors in because some of the viral proteins associated with EBV can enhance a cell’s signaling and help it survive.

Risk Factors for LRHL

There are some known risk factors associated with the development of LRHL and classical Hodgkin lymphoma in general. These risk factors include:

• Gender — Males are twice as likely to have it (compared to females).
• Family history — Risk level rises when family members (especially siblings) have had LRHL.
• EBV — A past infection with EBV (the virus that causes mononucleosis or “mono”) is common among those with LRHL.
• Immune system status — Having a weakened immune system increases someone’s risk.

Treatment for LRHL

Most cases of LRHL are detected at an early stage in the disease (stage 1 or 2). Early detection can lead to a better prognosis (outcome forecast) and better responses to treatment.

The treatment for LRHL generally consists of chemotherapy and radiation therapy. A common approach for classical Hodgkin lymphoma includes the chemotherapy regimen called ABVD.

Spelled out, ABVD includes the medications:

• Doxorubicin (Adriamycin or Rubex)
• Bleomycin (Blenoxane)
• Vinblastine (Velbane)
• Dacarbazine (DTIC-Dome)

Newer drugs that can also be used include:

• Pembrolizumab (Keytruda)
• Nivolumab (Opdivo)
• Brentuximab (Adcetris)

In cases that are more difficult to treat, a person with LRHL may undergo a stem cell transplant to replace their cancerous cells with healthy, noncancerous ones. This involves destroying cancerous blood cells with chemotherapy and replacing those with healthy new cells. In someone with a blood disease, a doctor transfers healthy stem cells into the person’s bone marrow or bloodstream. Healthy stem cells can come from the person undergoing treatment (autologous stem cell treatment) or from a donor (allogeneic stem cell transplants). To use someone with LDHL’s own stem cells, they must be collected before they undergo chemotherapy. The harvested matter then gets filtered to remove cancerous cells.

Connect With Others Who Understand

If you are living with lymphocyte-rich Hodgkin lymphoma (LRHL) — or any form of lymphoma — you are not alone. On MyLymphomaTeam, more than 11,000 members affected by lymphoma come together to ask questions, give advice, and share their experiences living with the condition.

Do you have LRHL? Share your experience in the comments below or start a conversation in MyLymphomaTeam.