National profile of the characteristics of patients treated for TTP an extension of the study: the characteristics of patients with TTP at the Universitas Haematology Department from 2010-2017 (HSD2018/0114)

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a rare life-threatening haematological disorder. It is caused by a deficiency in ADAMTS13. ADAMTS13 is a zinc-containing Metalloprotease enzyme that cleaves Von Willebrand factor (VWF). The deficiency of ADAMTS13 results in VWF accumulation and causes platelet rich thrombi, resulting in haemolytic anaemia and thrombocytopenia. There is an increased risk of TTP among female patients, patients from black races and patients with blood group O, based on previous studies. It is also most commonly seen among patients with HIV. The mainstay of treatment is plasma exchange and fresh frozen plasma. A retrospective study aimed At describing the national characteristics of TTP from 2010 to 2017 was done. Permission to conduct the study was obtained from the Health Sciences Research Ethics Committee of the University of the Free State (HSREC), Western Cape Blood Service (WCBS) ethics committee and South African National Blood Service (SANBS). The described characteristics are the age, gender, blood group and the blood products given to the patients.