Coagulation Changes in Children with Sickle Cell Anaemia during Painful Crises and Steady State at Federal Medical Centre Abeokuta, Nigeria

Abstract

Background: The pathophysiology of vaso-occlusive crises in sickle cell anaemia (SCA) is multifactorial and hypercoagulability is believed to play a role. The association between hypercoagulabilty and vaso-occlusive disease has been extensively studied in adult SCA patients, there is however paucity of data on the subject regarding paediatric SCA. Objective: This study set out to determine the presence of hypercoagulable states specifically in paediatric SCA subjects through quantification of specific coagulation markers during painful crises and steady state. Methodology: The study was a hospital- based longitudinal study carried out between May and October 2015 at Federal Medical Center, Abeokuta, Nigeria. Fifty SCA subjects were consecutively recruited during painful crises and followed up into their respective steady states. Twenty-five subjects with HbAA phenotype served as controls. Assays of coagulation markers, D-dimer and prothrombin fragment (F1 2) were carried out by sandwich ELISA method using MyBiosource？ D-dimer and F1 2 ELISA kits. Results: Mean D-dimer level was 7358 ± 4354.33 ng/ml in the SCA subjects during painful crises, 5509 ± 3506.2 ng/ml during steady state, and 800 ± 1874.14 ng/ml in HbAA controls. Mean (F1 2) level was 0.84 ± 0.43 nmol/l in the SCA subjects during painful crises, 0.64 ± 0.25 nmol/l during steady state, and 0.41 ± 0.28 nmol/l, in HbAA controls. The mean values of both coagulation markers assayed were significantly higher during painful crises than at steady state (P = 0.002), while steady state values were also significantly higher than that of haemoglobin AA individuals (P = 0.001). Conclusions: This study suggests the presence of hypercoagulable states in paediatric SCA during steady state which is exacerbated during painful crises. The clinical imports of this finding require further elucidation.