Nodular Lymphocyte Predominant Hodgkin Lymphoma

Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a type of Hodgkin disease, a cancer of lymph cells. It is one of the less frequent types of Hodgkin lymphoma, comprising about 5% of all cases of this cancer. However, it has several distinctive features that set it apart from other types of Hodgkin disease called the classical Hodgkin lymphomas.

Who Gets Nodular Lymphocyte Predominant Hodgkin Lymphoma?

NLPHL can occur at any age, although most patients are usually in their thirties. It is three times more common in males compared with the rate in females. There is no definitive cause, but risk factors include being infected with the Epstein-Barr virus and having a first-degree relative with Hodgkin lymphoma.

What Are the Symptoms of NLPHL?

The predominant warning sign of this disease is an enlargement of lymph nodes in the neck, chest, or armpits. Involvement of other lymph nodes or other organs is quite uncommon. Other symptoms of lymphoma include fever, weight loss, and sweating at night. A physical exam, medical history, and blood tests will be done.

The diagnosis of lymphoma is made with a lymph node biopsy. The exact type of lymphoma is determined by molecular tests that can tell the difference between NLPHL and other types of Hodgkin lymphoma. NLPHL is produced by disordered B-cells, which normally make up the part of the immune system that produces antibodies. They have a distinctive pattern of CD antigens, proteins on the surface of the cells, that allows for the diagnosis using biochemical analysis and CD marker assays. These unique CD antigen patterns also are used for targeted monoclonal antibody treatments.

How Is NLPHL Treated?

Sixty-three percent of patients with NLPHL are diagnosed with early-stage lymphom, involving lymph nodes only in the neck and upper half of the body. NLPHL tends to grow slower than classic Hodgkin lymphoma, and if you have no symptoms it may be decided to simply watch and wait for symptoms before starting treatment. If the lymphoma is in an early stage and you don't have any B symptoms it may be treated with radiation therapy only. B symptoms include fever, weight loss, and drenching night sweats.

If you have B symptoms or your case is at a more advanced stage, then treatment of nodular lymphocyte predominant Hodgkin lymphoma includes chemotherapy and often radiation therapy is done as well. The monoclonal antibody rituximab may also be given Most patients respond very well to treatment, and more than 90% are cured. Survival rates of Hodgkin disease are improving due to advances in treatment.

While this is a good prognosis, a 2010 study found that 10 years after treatment, 7% of NLPHL cases progressed to large B-cell lymphoma; after 20 years, the incidence increased to 30%. As NLPHL most often occurs in young men, it is important that they continue to be followed long-term to detect recurrence. While their initial treatment may seem to have produced a cure, the incidence of later relapse and progression is significant enough that they need continued monitoring long term.

Treatment of relapsed NLPHL patients with rituximab, a monoclonal antibody, is being explored.