Unfavorable presentation but comparable outcome: Presentation and outcome of children with nodular lymphocyte predominant Hodgkin lymphoma from India.

Background: Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is an uncommon subtype of Hodgkin lymphoma (HL) with few published studies in children, entirely from North America and Europe. We analyzed clinical features and treatment outcome of pediatric NLPHL.

Procedure: Children less than 18 years of age diagnosed after histopathology review to have NLPHL between June 1998 and August 2016 were retrospectively analyzed. Descriptive details of clinical presentation and treatment were collected, and outcomes analyzed using Kaplan-Meier survival analysis.

Results: Of the 42 patients with a confirmed diagnosis of NLPHL during this period, there was complete information on 35. Median age was 11 years (range 6-16 years), male:female ratio was 4.8:1, there were 15, 11, 6, and 3 patients with Stage I, Stage II, Stage III, and Stage IV disease, respectively. Six patients had B symptoms, 10 had bulky disease, and 3 had bone marrow as well as extranodal involvement. Histology was typical NLPHL in 23 and variant in 12. Twenty-nine received chemotherapy, 10 with additional radiation, 3 patients with early stage disease received only radiotherapy and three others underwent complete node resection alone. Median follow-up was 55 months (range 7-165 months), 5 year event-free survival (EFS) was 83.3%, and overall survival 97.1%. Variant NLPHL histology was associated with higher incidence of unfavorable presentation and lower EFS.

Conclusions: NLPHL in India has an excellent outcome, despite a higher incidence of unfavorable presentations such as advanced stage disease, B symptoms, and bulky disease. Variant histology is an adverse prognostic factor.