Nodular lymphocyte predominant Hodgkin lymphoma

Tumors generally located in the peripheral lymph nodes[11] which can be detected via PET scan and CT scan.

Ann Arbor staging is used to classify tumors and symptoms. Stage IV disease is very rare.[12]

There is a male predominance of NLPHL diagnosis.[7][13][12]

B symptoms may be uncommon.[7][3]

Diagnosis usually occurs at an early stage of disease progression.[7][13][12]

Watchful waiting (watch and wait) was defined as a period of observation of at least 3 months without any treatment.[16]

Surgical lymph node excision may be carried out at the time of diagnosis in certain cases such in children diagnosed at an early stage of progression.[13] One study found sustained complete remission in half of the cases with a watch-and-wait strategy after surgical lymph node excision at the time of diagnosis.[13]

Studies indicate that radiation therapy (radio therapy) may reduce the risk of progression in adults.[16][9] In one study, stage I-II patients treated with radiation therapy showed 10-year cause-specific survival of 98%, and the rate of developing radiotherapy-related second malignancies was not increased by the treatment (1% after 10 years).[17] A study published in 2013 on large group of patients with early-stage NLPHL indicated support for using limited-field radiation therapy as the sole treatment of early-stage disease.[9] In a study of 1,162 NLPHL patients from the Surveillance, Epidemiology and End Results (SEER) cancer registry program, radiation therapy improved overall survival and disease specific survival.[18]

An example antibody for use in immunotherapy is Rituximab. Rituximab has specific use in treatment of NLPHL as it is a chimeric monoclonal antibody against the protein CD20.[16] Studies indicate Rituximab offers potential in relapsed or refractory patients,[19] and also in front-line treatment[3] especially in advanced stages.[13] Because of a tendency for relapse, maintenance treatment such as every 6 months for 2 years is suggested.[12] Rituximab has been shown to improve patient outcomes after histological transformation.[20]

Possible options such as anthracycline-containing regimens include ABVD, BEACOPP and CHOP.[16] Results of a trial with COPP/ABV in children suggested positive results with chemotherapy alone are possible without the need for radiation therapy.[21] Optimal chemotherapy is a topic for debate, for example there is evidence of support for treatment with R-CHOP instead of ABVD, results showing high rates (40%) of relapse after 10 years since ABVD chemotherapy.[12] BEACOPP has higher reported toxicity risk.[22]

One study reported combined radiation therapy (radio therapy) and antibody Rituximab.[16] R-CHOP optionally followed by radiation therapy is recommended in newly diagnosed late stage disease, while for early stage disease radio therapy alone (stage IA without risk factors) or a brief ABVD-based chemotherapy followed by radiation therapy (early stages other than stage IA without risk factors) was advised.[22]

One study in the United States has suggested improved overall survival in response to chemotherapy for African Americans.[23]

Histologic transformation to diffuse large B-cell lymphoma (DLBCL) can occur in up to 12% of cases.[13] After transformation, neoplastic cells carry monoclonal immunoglobulin gene rearrangements.[13] Histological transformation may lead to poor prognosis[12] and therefore repeat biopsy is required at relapse.[13] One study found a transformation rate of 7.6%, and suggested that prior exposure to chemotherapy and a presentation with splenic involvement were associated with increased risks of transformation.[20]