Relapsed or poorly responsive nodular lymphocyte predominant Hodgkin lymphoma in children and adolescents – a report from the United Kingdom's Children's Cancer and Leukaemia Study Group

Abstract

There is a paucity of data on the treatment outcome in children with relapsed or poorly responsive nodular lymphocyte predominant Hodgkin lymphoma (nLPHL). This retrospective report evaluates the treatment outcome in a national cohort of children with relapsed or poorly responsive nLPHL. A total of 37 patients, 22 with relapsed and 15 with poorly responding disease, are the subjects of this report. Of the 22 patients with relapsed nLPHL, 11 had relapsed after primary excision biopsy, 10 after chemotherapy and 1 after chemotherapy and involved field radiotherapy. The majority had localized disease at relapse. The median time to relapse was 8 months after chemotherapy and 11 months after excision biopsy. Seven of the 15 patients with poorly responding nLPHL had variant histology. Three patients with initial poor response did not receive any further treatment and have had no disease progression. Transformation to diffuse large B cell lymphoma, in addition to evolution from typical to variant nLPHL occurred in one patient each. Thirty-four patients have been successfully re-treated with second chemotherapy or radiotherapy. Multiple relapses were uncommon but treatable. Relapse or poorly responsive nLPHL is fully salvageable with either additional chemotherapy and or radiotherapy.

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

“…European cohorts have noted an increased incidence of advanced stage disease, poorer response to chemotherapy, and increased recurrences in variant histology NLPHL as compared to patients with typical NLPHL, although the influence on overall survival is inconsistent 26,30,32,[35][36][37][38][39][40]. Our study also noted the higher incidence of unfavorable presentation and poorer EFS in children with variant NLPHL histology.…”

“…Excellent overall survivals have been reported with complete nodal excision of Stage IA (especially when only a single node was involved), involved field radiotherapy alone in Stage IA disease, or lower intensity chemotherapy in early stage unresectable disease . Relapses in NLPHL have been found amenable to salvage chemotherapy, hence the trend toward decreasing the intensity of frontline therapy to minimize the risk of treatment toxicity and late effects . Rituximab has shown potential benefit in frontline as well as salvage treatment in adult NLPHL, however it is yet to be studied in pediatric NLPHL .…”

“…[2][3][4][5][6][7][8]20,24,30 In our cohort of 35 [4][5][6]20,21,23,24 Relapses in NLPHL have been found amenable to salvage chemotherapy, hence the trend toward decreasing the intensity of frontline therapy to minimize the risk of treatment toxicity and late effects. [18][19][20][21]32 Rituximab has shown potential benefit in frontline as well as salvage treatment in adult NLPHL, however it is yet to be studied in pediatric NLPHL. 33 19 Treatment of children with advanced stage disease is controversial, with most groups recommending treatment with multiagent chemotherapy (with or without rituximab) as in classical Hodgkin lymphoma.…”

Nodular lymphocyte predominant Hodgkin lymphoma: pathology, clinical course and relation to T-cell/histiocyte rich large B-cell lymphoma

“…88 Good results with conventional chemotherapy as a salvage approach were also obtained from a retrospective study including children and adolescents with NLPHL recurrence or poor response to the initial treatment. 93 Conventional chemotherapy optionally combined with RT and/or anti-CD20 antibody treatment therefore should be considered as salvage therapy in patients who had no or little chemotherapy as part of their first-line treatment.…”

Variant histology nodular lymphocyte predominant Hodgkin lymphoma – a route to transformation?

“…nLPHL is an indolent lymphoma and aggressive clinical behaviour is rare at presentation and relapse (Shankar et al , ). The unusual histological evolution from typical nLPHL to the diffuse T cell rich variant with accompanying aggressive clinical progression in this patient resembles the high‐grade transformation seen in low‐grade B cell non‐Hodgkin lymphomas, such as follicular lymphoma.…”