Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL)

Although treatment of adult patients with NLPHL has traditionally involved high-dose radiation alone, treatment of children originally involved chemotherapy plus LD-ISRT. Standard of care in pediatric NLPHL at present is unclear but may include chemotherapy alone or, for limited disease, complete resection of isolated nodal disease without chemotherapy. Surgical resection of localized disease produces a prolonged disease-free survival in a substantial proportion of patients, obviating the need for immediate cytotoxic therapy.[27,28,33,34] Even if cytotoxic therapy is required, the possibility of avoiding chemotherapy and radiation in prepubertal children is advantageous.

Results from a single-arm Children's Oncology Group (COG) trial support the strategy of observation after surgical resection of a single node and treatment with limited chemotherapy for children with favorable stage IA or IIA NLPHL.[27][Level of evidence B1] To date, there is no evidence that this approach increases the risk of transformation to non-Hodgkin lymphoma.

Results from a single-arm Children's Oncology Group (COG) trial support the strategy of observation after surgical resection of a single node and treatment with limited chemotherapy for children with favorable stage IA or IIA NLPHL.[27][Level of evidence B1] To date, there is no evidence that this approach increases the risk of transformation to non-Hodgkin lymphoma.

A total of 178 patients were treated with surgical resection alone for single-node disease (n = 52), chemotherapy alone after complete response (CR) to three cycles of doxorubicin, vincristine, prednisone, and cyclophosphamide (AV-PC) (n = 115), or chemotherapy with low-dose involved-field radiation therapy (LD-IFRT) (21 Gy) after incomplete response to AV-PC chemotherapy (n = 11). The 5-year EFS rate was 85.5%, and the OS rate was 100%.

Results from a single-arm Children's Oncology Group (COG) trial support the strategy of observation after surgical resection of a single node and treatment with limited chemotherapy for children with favorable stage IA or IIA NLPHL.[27][Level of evidence B1] To date, there is no evidence that this approach increases the risk of transformation to non-Hodgkin lymphoma.

The 5-year EFS rate was 77% for patients observed after total resection and 88.8% for patients treated with AV-PC chemotherapy.

In a retrospective review of 41 patients with advanced-stage NLPHL, many different chemotherapy regimens were used; some included rituximab.[35][Level of evidence C1]

In a retrospective review of 41 patients with advanced-stage NLPHL, many different chemotherapy regimens were used; some included rituximab.[35][Level of evidence C1]

The OS rate was 98%, with the only death resulting from a subsequent neoplasm.

In a retrospective analysis, 97 intermediate-risk patients with NLPHL were treated in COG study AHOD0031 (NCT00025259).[36]

In a retrospective analysis, 97 intermediate-risk patients with NLPHL were treated in COG study AHOD0031 (NCT00025259).[36]

These patients demonstrated a higher CR rate than patients with classical histology. The 5-year EFS rate was marginally superior in patients with NLPHL (91.2%) than in patients with classical Hodgkin lymphoma (83.2%).

In a retrospective analysis, 97 intermediate-risk patients with NLPHL were treated in COG study AHOD0031 (NCT00025259).[36]

Most patients treated with four cycles of the ABVE-PC regimen achieved a rapid early response with a CR status and demonstrated excellent EFS and OS without IFRT. This finding suggests that the dose-dense, response-based protocol therapy designed for patients with classical Hodgkin lymphoma may have been more intensive than necessary for patients with NLPHL.