| Summary: | Introduction: Multiple cause of death methodology enhances mortality studies beyond the traditional underlying cause of death approach. Aim: This study aims to describe causes of death and mortality issues related to haemophilia with the use of multiple-cause-of-death methodology. Methods: Annual male haemophilia mortality data was extracted from the public multiple-cause-of-death databases of the Mortality Information System, searching deaths included in rubrics D66 “hereditary factor VIII deficiency” (haemophilia A), and D67 “hereditary factor IX deficiency” (Haemophilia B) of the International Classification of Diseases, Tenth Revision, and processed by the Multiple Cause Tabulator. Results: In Brazil, from 1999 to 2016, a total of 927 male deaths related to haemophilia occurred during the 18 year period, of which 418 (45,1 %) as underlying cause, and 509 (54,9 %) as associated cause of death. The leading associated cause of 418 deaths of haemophilia as underlying cause was hemorrhage (52.6%), half of which intracranial hemorrhage. Infectious and parasitic diseases accounted for 40,5% as the underlying causes of 509 deaths where haemophilia was an associated cause, where human immunodeficiency virus disease prevailed, however falling from 37,0% to 19.7%, and viral hepatitis increased from 6.0% to 7.9%; diseases of the circulatory system, increased from 13.5% to 18.4%, including intracranial hemorrhage from 5.7% to 7.0%, and neoplasms, from 8,5% to 13.2%, respectively from 1999-2007 to 2008-2016, followed as main underlying causes. Conclusion: Hemorrhages, mainly intracranial hemorrhage, human immunodeficiency virus disease, and viral hepatitis are the chief prevention goals aiming at the control of haemophilia mortality.
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