title: Malignancies in Swedish persons with haemophilia: a longitudinal registry study.
abstract: The aim of the study was to investigate, over time, the incidence of and mortality due to malignant diseases among persons with haemophilia, compared to matched controls. Persons with haemophilia A or B were enrolled via registries at each haemophilia centre, as well as from the National Patient Registry, and were compared to five sex and age-matched controls per patient. Data from the national Cancer Registry were linked to the study participants. A total of 1431 persons with haemophilia and 7150 matched controls were enrolled. Between the years 1972 and 2008, 164 malignancies were reported. The most common type of cancer among patients was prostate cancer, followed by haematologic malignancies, including lymphoma and leukaemia, which were significantly more frequent in patients [n = 35 (2.4%) vs. n = 60 (0.8%); P < 0.001]. Malignancies in bladder and other urinary organs were also significantly different [n = 21 (1.5%) vs. n = 46 (0.6%); P < 0.01]. The overall incidence rate ratio of malignancies per 1000 person-years compared to the controls was 1.3 [95% confidence interval (CI) 1.1, 1.6]. In subgroup analysis, the corresponding incidence rate ratios per 1000 person-years for persons with severe haemophilia was 1.7 (95% CI 0.9, 3.1) and that for mild/moderate haemophilia 1.1 (95% CI 0.8, 1.5). Swedish persons with haemophilia had a significantly higher incidence of malignant diseases than controls. These were primarily haematologic malignancies and cancer in urinary organs, and the difference independent of any co-infections with HIV and/or viral hepatitis. The findings indicate the importance of further studies and close follow-up of malignancies in persons with haemophilia.
authors.affiliation: aDepartment of Clinical Sciences, Clinical Coagulation Research Unit, Lund University, Skåne University Hospital, Malmö bCardiovascular Epidemiology, Department of Medical Science, Uppsala University Hospital, Uppsala cAstraZeneca, Department of Epidemiology, R&D, Mölndal dCoagulation Centre, Department of Medicine/Hematology and Coagulation Disorders, Sahlgrenska University Hospital, Gothenburg eCoagulation Unit, Hematology Centre, Karolinska University Hospital and Department of Medicine, Karolinska Institute, Stockholm fDepartment of Hematology and Coagulation Disorders, Skåne University Hospital, Malmö, Sweden.
authors.forename: Susanna
authors.initials: S
authors.lastname: Lövdahl
authors.forename: Karin M
authors.initials: KM
authors.lastname: Henriksson
authors.forename: Fariba
authors.initials: F
authors.lastname: Baghaei
authors.forename: Margareta
authors.initials: M
authors.lastname: Holmström
authors.forename: Erik
authors.initials: E
authors.lastname: Berntorp
authors.forename: Jan
authors.initials: J
authors.lastname: Astermark
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016022:Case-Control Studies; D002648:Child; D005260:Female; D019337:Hematologic Neoplasms / Q000150:complications / Q000453:epidemiology* / Q000473:pathology; D006467:Hemophilia A / Q000150:complications / Q000453:epidemiology* / Q000473:pathology; D002836:Hemophilia B / Q000150:complications / Q000453:epidemiology* / Q000473:pathology; D006801:Humans; D015994:Incidence; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D011471:Prostatic Neoplasms / Q000150:complications / Q000453:epidemiology* / Q000473:pathology; D012042:Registries; D012720:Severity of Illness Index; D013548:Sweden / Q000453:epidemiology; D001749:Urinary Bladder Neoplasms / Q000150:complications / Q000453:epidemiology* / Q000473:pathology