Treatment options for nodular lymphocyte predominant Hodgkin lymphoma

The current article reviewed the treatment options for nodular lymphocyte predominant Hodgkin lymphoma. The article highlighted standard treatment options as well as the experience of one medical center.

Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is an uncommon type of Hodgkin lymphoma. It occurs in roughly 5% of Hodgkin lymphomas. Because it occurs in such a small number of patients, few studies have examined it directly. Most patients, however, have long overall survival (time from treatment until death from any cause). One goal in choosing treatments is to minimize long-term toxicity (negative effects). Treatment options are often based on what is known to be effective in other forms of Hodgkin lymphoma. 

The current study reviewed what is known about treatment options for NLPHL.

NLPHL is often diagnosed in the early stages. Early-stage NLPHL generally has a good prognosis. Two studies have noted that age over 45, disease stage, and the presence of symptoms like night sweats, fever, and weight loss were associated with a worse overall survival (time from treatment until death from any cause). Treatment for early-stage NLPHL usually includes radiation alone. This differs from other forms of Hodgkin lymphoma, where chemotherapy combined with radiation is the standard treatment. NLPHL patients with risk factors for poor prognosis may be treated with the combined treatment.

Advanced stage NLPHL (stages III or IV or those that have risk factors) is associated with a poorer prognosis. Treatment includes chemotherapy and rituximab (Rituxan). Chemotherapy is the main treatment option. The most effective combination is still under investigation. It has been suggested that alkylating agents (such as mechlorethamine or cyclophosphamide) are more effective in NLPHL. One study compared three chemotherapy combinations, two of which included mechlorethamine. Rate of relapse was 32% in those treated with that agent, compared to 75% in those treated with another combination (ABVD, doxorubicin, bleomycin, vinblastine, and dacarbazine). Another study examined RCHOP (rituximab, cyclophosphamide, adriamycin, vincristine, and prednisone). After an average of 42 months, none of the 15 patients had relapsed.

Rituximab is a monoclonal antibody. This type of treatment works against cells that express (produce) the protein CD20. Rituximab binds with and kills the cancer cells. This treatment is used in combination with chemotherapies. There is a high response rate to this treatment, but patients often relapse.

In patients who have relapsed, some combination of chemotherapy, radiation and rituximab is recommended, as well as stem cell transplant. Few studies have examined survival in relapsed NLPHL. One small study reported a 76% five-year survival rate following high-dose chemotherapy and stem cell transplant. Treatment decisions often depend on tumor size and symptoms experienced. 

This study highlighted the most effective treatments currently known for NLPHL, and suggested that further research is needed.