Pediatric lymphocyte-predominant Hodgkin lymphoma: Review of Spanish patients between November-2007 and October-2019

Introduction From 2007 SEHOP Hodgkin Lymphoma Working Group decided to recommend pediatric patients(p) diagnosed with lymphocyte-predominant Hodgkin lymphoma (LPHL) were treated according to Euronet-PHL-LP1 for stage IA and IIA and according to updated international strategies for advanced stages. From 2008 pathology central review was encouraged taking advantage of the Euronet-C1 trial pathology reviewers. Objective: Collect and review outcome of pediatric p withLPHL diagnosed in Spain 2007–2019.

Methods Questionnaire was sent to 36 Pediatric Onco-Hematology Units. Descriptive analysis was performed: sex distribution, mean age at diagnosis, pathology central review, PET, stage, treatment, median follow-up, outcome and relapse treatment.

Results 28 out of 36 Pediatric Onco-Hematology Units responded the questionnaire:52p from 26 hospitals with LPHL were collected. Gender distribution:34 male/18 female(1.88/1). Mean age at diagnosis: 10 years(yrs) and 6 months(m)(5-16yrs). Centralized pathological review was performed in 61,5%. PET was used for staging in 80,7%. Stage distribution: IA 18p,IIA 22p,IIB 1p,IIIA 6p,IV 5p. Most frequent location: Cervical.

Treatment:15/18p stage I underwent surgery alone;11/22p stage IIA received 3 CVP and 5/22 3 R-CVP,2/22 combination of CVP and R-CVP,4/22 received polichemotherapy. Advanced stages were treated according to Euronet-C1 or LH-2003 trials or with R-CHOP.

Outcome: Median follow up 49m(range 3m-132m).13/52p (25%) relapsed with median relapse time of 9m(5m-52m).Stage I:5/15p(33,3%) treated with surgery alone relapsed,median 7m(range 5-9m),IIA: 4,III: 2 and IV 2p. Treatment: stage I polychemotherapy (CVP,R-CVP) IIA-IV:IEP/ABVD/ICE +rituximab + radiotherapy: 1IV. 4p underwent TPH.All 52p p are alive: 38p in 1^st complete remission(CR),12 in 2^nd CR,1 in PR still on chemotherapy,1 in 3^rd CR after R-CHOP and radiotherapy.

Conclusion In our series,Spanish p with LPHL have an excellent prognosis. Increasing centralized pathology review along last decade has been a major achievement to improve the quality of pathology diagnosis. Surgery alone was effective treatment for 66% of stage I p. 79% of p treated with low intensity chemotherapy or polychemotherapy + rituximab remain in 1st CR. Relapsed p can be safely rescued with low intensity chemotherapy and polychemotherapy +rituximab + radiotherapy. Unified international recommendations, participation in international multicenter trials and central pathology review will continue to be encouraged for Spanish pediatric p with LPHL along next years.

Conflict of Interest:

A Fernández-Teijeiro has had a consulting or advisory role for Amgen, Novartis, Takeda, SOBI and Bayer. She has has received honoraria from Takeda and Amgen for educational events and travel expenses from Servier, Shire and Gilead.