ADAMTS13 levels in sickle cell anaemia: is there a change between steady-state and vaso-occlusive crisis?

Abstract

Background Sickle cell anaemia (SCA) is a hypercoaguable state characterized by a significant alternation in haemostatic parameters which may predispose to increased risk of vas-occlusive crisis (VOC). The role of ADAMTS13 in the pathogenesis of VOC has not been fully investigated in our environment. Objective The objective of this study is to evaluate the level of ADAMTS13 in SCD subject in Calabar and to determine their role in the pathogenesis of vaso-occlusive crisis. Materials and methods This is a comparative study carried out at the University of Calabar Teaching Hospital (UCTH), Calabar. In all, 60 SCA patients were evaluated in VOC and steady states well are 50 apparently healthy controls. ADAMTS13 Ag was evaluated using Assaypro enzyme-linked immunosorbent assay kits with LOT Nos 04222167 R. Data were analyzed with IBM SPSS Chicago software version 21. The study was approved by the UCTH Institution Ethical Review Board. Results The mean ages of the SCA subjects and controls were 23.5 + 7.2 yrs and 26.5 + 5.6 years respectively (P=0.706). There were 23 (38.3%) males in the SCA group and 21 (42.0%) females in the controls. There was no significant difference in their sex distribution (P=0.063). The mean (SD) of ADAMTS13 in VOC, steady-state, and controls were 0.61 + 0.10 ug/l, 0.44 + 0.06 ug/l, and 0.62 + 0.10 ug/l respectively. ADAMTS13 levels did not differ significantly across the groups (P=0.270). Conclusion ADAMTS13 level is not elevated both in steady-state and vaso-occlusive crisis