title: A 20-year population-based study on the epidemiology, clinical features, treatment, and outcome of nodular lymphocyte predominant Hodgkin lymphoma.
abstract: Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) is a subtype of Hodgkin lymphoma characterized by a unique clinical and histological presentation. Because of the rare nature of this disease, few large-scale studies are available. We conducted a cohort study in which patients were identified in the Netherlands Cancer Registry in the Southeast of the Netherlands between 1990 and 2010. Of these patients, we collected all clinical characteristics and re-reviewed pathologic material to confirm NLPHL diagnosis. Seventy-three histologically confirmed cases of NLPHL were analyzed with a median follow-up of 65 months (range 4-257 months). Median age at diagnosis was 43 years (range 1-87); 84.9 % of the patients were male; B symptoms were present in 5.5 %; and stage I/II disease was most common (75.4 %). Patients were primarily treated with radiotherapy (50.7 %), chemotherapy (26 %), combined modality (radiotherapy and chemotherapy) (11 %), or surgical excision with careful watch-and-wait (12.3 %). Relapses occurred in seven patients (9.6 %) after a median of 26 months (21-74 months). Six patients (8.2 %) developed histologic transformation to large cell lymphoma. Five patients (6.8 %) died during follow-up due to progression of NLPHL (n = 1), histologic transformation (n = 2) and intercurrent deaths (n = 2). The estimated 10-year overall survival was 94.0 % and the 10-year progression-free survival 75.8 %. Our study confirms the distinct characteristics of NLPHL with a relatively good long-term prognosis. It may be possible to reduce treatment intensity in early stage NLPHL without affecting long-term outcome.
authors.affiliation: Department of Hematology, Radboud University Medical Center, Geert Grooteplein 8, 6525 GA Nijmegen, Postbus 9101, 6500 HB, Nijmegen, The Netherlands. l.strobbe@gelre.nl.
authors.forename: L
authors.initials: L
authors.lastname: Strobbe
authors.affiliation: Department of Hematology, Radboud University Medical Center, Geert Grooteplein 8, 6525 GA Nijmegen, Postbus 9101, 6500 HB, Nijmegen, The Netherlands.
authors.forename: L L F G
authors.initials: LL
authors.lastname: Valke
authors.affiliation: Department of Hematology, Radboud University Medical Center, Geert Grooteplein 8, 6525 GA Nijmegen, Postbus 9101, 6500 HB, Nijmegen, The Netherlands.
authors.forename: I J
authors.initials: IJ
authors.lastname: Diets
authors.affiliation: Department of Pathology, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: M
authors.initials: M
authors.lastname: van den Brand
authors.affiliation: Department of Registry and Research, Comprehensive Cancer Center, Utrecht, The Netherlands.
authors.forename: K
authors.initials: K
authors.lastname: Aben
authors.affiliation: Department of Hematology, Radboud University Medical Center, Geert Grooteplein 8, 6525 GA Nijmegen, Postbus 9101, 6500 HB, Nijmegen, The Netherlands.|Department of Hematology, Rijnstate Hospital, Arnhem, The Netherlands.
authors.forename: J M M
authors.initials: JM
authors.lastname: Raemaekers
authors.affiliation: Department of Pathology, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: K M
authors.initials: KM
authors.lastname: Hebeda
authors.affiliation: Department of Pathology, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: J H J M
authors.initials: JH
authors.lastname: van Krieken
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006689:Hodgkin Disease / Q000175:diagnosis* / Q000401:mortality* / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009426:Netherlands / Q000453:epidemiology; D011159:Population Surveillance / Q000379:methods; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
keywords: Chemotherapy; Epidemiology; Nodular lymphocyte predominant Hodgkin lymphoma; Outcome; Radiotherapy