Advanced stage nodular lymphocyte predominant Hodgkin lymphoma in children and adolescents: clinical characteristics and treatment outcome – a report from the SFCE & CCLG groups

Abstract

Advanced stage nodular lymphocyte predominant Hodgkin lymphoma (nLPHL) is extremely rare in children and as a consequence, optimal treatment for this group of patients has not been established. Here we retrospectively evaluated the treatments and treatment outcomes of 41 of our patients from the UK and France with advanced stage nLPHL. Most patients received chemotherapy, some with the addition of the anti CD20 antibody rituximab or radiotherapy. Chemotherapy regimens were diverse and followed either classical Hodgkin lymphoma or B non-Hodgkin lymphoma protocols. All 41 patients achieved a complete remission with first line treatment and 40 patients are alive and well in remission. Eight patients subsequently relapsed and 1 patient died of secondary cancer (9 progression-free survival events). The median time to progression for those who progressed was 21 months (5·9-73·8). The median time since last diagnosis is 87·3 months (8·44-179·20). Thirty-six (90%), 30 (75%) and 27 (68%) patients have been in remission for more than 12, 24 and 36 months, respectively. Overall, the use of rituximab combined with multi-agent chemotherapy as first line treatment seems to be a reasonable therapeutic option.

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.…”

“…The median age at presentation of 11 years in our study corroborates well with published literature, as did the marked male predominance. [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.…”

Outcomes in intermediate‐risk pediatric lymphocyte‐predominant Hodgkin lymphoma: A report from the Children's Oncology Group

“…Although there is no consensus on the optimal chemotherapy‐only regimen for children with LPHL, several studies have utilized chemotherapy regimens designed to minimize anthracycline and alkylator exposure . The Children's Cancer and Leukaemia Group (CCLG) and the Société Française de lutte contre les Cancers et leucémies de l'Enfant et l'adolescent (SCFE) recently reported on the outcomes of a retrospective series of 41 pediatric patients with advanced stage LPHL (clinical stage IIB, III, and IV) treated with diverse regimens . Twenty patients received chemotherapy alone with either cHL or B‐cell non‐Hodgkin lymphoma (NHL) protocols and all attained a CR.…”

“…Three patients subsequently relapsed but had a CR to second‐line therapy. Chemotherapy regimens varied and included doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD)/ChIVPP ( n = 7) and cyclophosphamide, vincristine, and prednisone (CVP) ( n = 3) as well as rituximab ( n = 12) in combination with other chemotherapy, most commonly cyclophosphamide, doxorubicin, vincristine, prednisone (R‐CHOP) ( n = 5) or R‐ABVD ( n = 3) . Although numbers were small, the rituximab treatment group was notable for having a lower relapse rate of 8.3% (1/12) compared with the chemotherapy alone group rate of 15% (3/20).…”

Use of flow cytometry in the phenotypic diagnosis of hodgkin's lymphoma

“…They express B cell markers such as CD20+, CD79a, Pax5, Oct-2, Bob-1, and J chain. CHL markers CD30 and CD15 are negative (5)(6)(7). EBV is rarely expressed by these cells.…”