Mortality of people with inherited bleeding disorders in a tertiary academic centre

Ganwo, Ibrahim
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Background. Inherited bleeding disorders (IBDs) are a heterogeneous group of clinical conditions that include abnormalities of blood vessels, coagulation proteins or platelets present at birth. Haemophilia A and B are the major inherited bleeding disorders with the high mortality rate in comparison with the other inherited bleeding disorders. Owing to the availability of virally inactivated plasma-derived clotting factor concentrates (pCFC) and the introduction of combined antiretroviral therapy (cART) for all people with HIV (human immunodeficiency virus) infection, the mortality rate and the leading cause(s) of death in people with IBDs were dramatically changed from bleeding and HIV/AIDS (acquired immunodeficiency syndrome) related deaths to other commonly known causes of mortality such as trauma, cancer and diabetes. There are currently no documented data from South Africa detailing the specific cause(s) of mortality in IBDs. Objectives. The main aim of this study is to document the cause(s) of death in a cohort of South African people with inherited bleeding disorders from a single tertiary centre and to evaluate the role of co-morbidities in contributing to their mortality. Methods. The study was a single-centre retrospective cohort study performed at Charlotte-Maxeke Johannesburg Academic Hospital Haemophilia Comprehensive Care Centre (CMJAH-HCCC). Key inclusion criteria were any person with an inherited bleeding disorder with complete hospital records regardless of age. Study procedures included extraction of anonymised data from each patient’s file onto a data collection sheet. Results. Complete data sets were available on 73 people with IBDs who died between 1990 and 2016. The majority of the population was male and Caucasians with a mean age of 38 years. The crude mortality rate for the whole cohort was 10.4% per 100 person-years. The overall mortality rate of people with haemophilia (A and B) was 13.8% while the crude mortality rate of people with Von Willebrand Disease (VWD) was 3.1% with higher mortality rates with type II and type III VWD compared to type I (7.1%, 5.2% vs 2.4% respectively). In this cohort, HIV/AIDS was the leading cause of death (45%) in the 1990s which markedly decreased in the last two decades (<7% in 2000s and <1% 2010s). In the last decade, spontaneous and traumatic bleeding episodes were now the leading causes of death (20%), with intracranial haemorrhage causing 57% of haemorrhagic deaths. Conclusions. This retrospective study showed a decrease in cause-related mortality in people with IBD in the last three decades. Bleeding is now the leading cause of mortality in this population
A research report (in the format of a “submissible” paper) submitted to the Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, in partial fulfillment of the requirements for the degree of Master of Medicine in the branch of Haematology. Johannesburg, 2019