Latest guidelines relating to the treatment of the nodular lymphocyte predominant Hodgkin lymphoma.

This review provided guidelines relating to the treatment of nodular lymphocytic-predominant Hodgkin lymphoma (NLPHL). The authors concluded that appropriate treatment varied by age, disease-stage and the presence of relapse.

NLPHL is the rarer form of HL. It is characterized by the presence of atypical white blood cells called popcorn cells. These cells have CD20 (a protein) on their surface. NLPHL accounts for 3-8% of all HL in adolescents and adults, and 10-20% in younger children. There is currently only a small number of studies relating to the treatment of NLPHL. The British Committee for Standards in Hematology brought together a panel of experts to provide recommendations for the treatment of NLPHL.

This review compiled and evaluated the most up-to-date evidence on the treatment of NLPHL.

ll NLPHL patients regardless of disease stage should consider participating in a clinical trial.

Early-stage patients include those with Stage I and Stage II disease. Surgery should remove localized disease when possible. Those with any remaining localized disease should be treated with involved-field (targeted) radiation therapy (IFRT). Some patients with stage 2A disease are not able to be treated with IFRT. These patients may have more than two tumor sites, or tumors close to organs such as the kidneys, bowels, or pancreas. These patients should be treated like advanced-stage patients.

Advanced-stage patients include those with Stage III and Stage IV disease. The recommended combined chemotherapies are R-CVP (rituximab, cyclophosphamide, vincristine, prednisolone); R-CVinbP (rituximab, cyclophosphamide, vinblastine, prednisolone); and R-ABVD (rituximab, doxorubicin, bleomycin, vinblastine, dacarbazine). The monoclonal antibody (a treatment that attaches to cancer cells, killing them or making them unable to grow) rituximab (Rituxan) should be recommended in patients who are not able to tolerate combination chemotherapy.

Relapsed-disease patients should be screened to see if the disease has transformed into another form of lymphoma. In patients with isolated local re-occurrence radiation is recommended. Advanced-stage patients who do not have any symptoms (such as fever, night sweats, or weight loss) should watch and wait rather than be treated. Patients who are symptomatic should be treated with salvage (second-line) chemotherapy. Patients with other serious conditions should consider rituximab. 

Special considerations apply to children and adolescents under 19. Stage I patients who have had their cancer surgically removed should not be treated with chemotherapy. Patients with early-stage disease that cannot be surgically removed should be treated with low-dose chemotherapy (LDC) without anthracyclines (such as doxorubicin). For advanced-stage patients rituximab or R-CHOP (rituximab, cyclophosphamide, doxorubicin hydrochloride, vincristine, prednisolone) is recommended. Treatment options for patients who have relapsed for the first time should take disease stage, the time to relapse, and the age at relapse into consideration. Treatment options include surgery, LDC and / or radiotherapy. Those with multiple-relapsed disease should be treated with a combination of rituximab and chemotherapy.

The authors concluded that the treatment of patients with NLPHL depends on age, disease stage and the presence of relapse.