title: Risk of familial classical Hodgkin lymphoma by relationship, histology, age, and sex: a joint study from five Nordic countries.
abstract: We aimed to provide the familial risk of classical Hodgkin lymphoma (HL) by relationship, histology, age at diagnosis, and sex. A cohort of 57,475 first-degree relatives of 13,922 HL patients diagnosed between 1955 and 2009 in 5 European countries was observed for HL incidence. The overall lifetime cumulative risk (CR) of HL in first-degree relatives of a patient with HL was 0.6%, which represents a threefold (standardized incidence ratio [SIR], 3.3; 95% confidence interval [CI], 2.8-3.9) increased risk over the general population risk. The risk in siblings (6.0-fold; 95% CI, 4.8- to 7.4-fold) was significantly higher than in parents and/or children (2.1-fold; 95% CI, 1.6- to 2.6-fold). Very high lifetime risk of HL was found for those with multiple affected first-degree relatives (13-fold; 95% CI, 2.8- to 39-fold) and for same-sex twins (57-fold; 95% CI, 21- to 125-fold). We found high familial risks between some concordant histologic subtypes of HL such as lymphocyte-rich (81-fold; 95% CI, 30- to 177-fold) and nodular sclerosis (4.6-fold; 95% CI, 2.9- to 7.0-fold) and also between some discordant subtypes. The familial risk in sisters (9.4-fold; 95% CI, 5.9- to 14-fold) was higher than in brothers (4.5-fold; 95% CI, 2.9- to 6.7-fold) or unlike-sex siblings (5.9-fold; 95% CI, 4.3- to 8.1-fold). The lifetime risk of HL was higher when first-degree relatives were diagnosed at early ages (before age 30 years). This study provides tangible absolute risk estimates for relatives of HL patients, which can be used as a sex-, age-, and family history-based risk calculator for classical HL by oncologists and genetic counselors.
authors: Division of Molecular Genetic Epidemiology, German Cancer Research Center, Heidelberg, Germany;
authors: Elham
authors: E
authors: Kharazmi
authors: Division of Molecular Genetic Epidemiology, German Cancer Research Center, Heidelberg, Germany; Division of Preventive Oncology, National Center for Tumor Diseases (NCT), Heidelberg, Germany;
authors: Mahdi
authors: http://orcid.org/0000-0002-6639-065X
authors: M
authors: Fallah
authors: Finnish Cancer Registry, Institute for Statistical and Epidemiological Cancer Research, Helsinki, Finland; School of Health Sciences, University of Tampere, Tampere, Finland;
authors: Eero
authors: E
authors: Pukkala
authors: Danish Cancer Society Research Center, Copenhagen, Denmark;
authors: Jörgen H
authors: JH
authors: Olsen
authors: Icelandic Cancer Registry, Reykjavik, Iceland; Faculty of Medicine, University of Iceland, Reykjavik, Iceland;
authors: Laufey
authors: L
authors: Tryggvadottir
authors: Center for Primary Health Care Research, Lund University, Malmö, Sweden; and.
authors: Kristina
authors: K
authors: Sundquist
authors: Norwegian Cancer Registry, Oslo, Norway.
authors: Steinar
authors: S
authors: Tretli
authors: Division of Molecular Genetic Epidemiology, German Cancer Research Center, Heidelberg, Germany; Center for Primary Health Care Research, Lund University, Malmö, Sweden; and.
authors: Kari
authors: K
authors: Hemminki
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D020022:Genetic Predisposition to Disease; D006689:Hodgkin Disease / Q000453:epidemiology / Q000235:genetics* / Q000473:pathology*; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D011379:Prognosis; D012042:Registries; D018570:Risk Assessment; D012537:Scandinavian and Nordic Countries / Q000453:epidemiology; D012737:Sex Factors; D055815:Young Adult