A STUDY OF THE CO-RELATIONSHIP BETWEEN PROTEIN C LEVELS AND COMPLICATIONS OF SICKLE CELL DISEASE IN UNIVERSITY OF BENIN TEACHING HOSPITAL

Abstract

BACKGROUND: Sickle cell disease (SCD) is characterized by vaso-occlusive events and organ
damage which form important causes of morbidity and mortality. Although protein C, a naturally
occurring anticoagulant has been demonstrated to be deficient in sickle cell patients, the impact of
hypercoagulable states and thrombosis on sickle cell disease still remains uncertain.
OBJECTIVE: The objective of this study was to determine the protein C (PC) levels of the sickle cell
patients and to explore the relationship between protein C levels and vaso-occlusive events as well as
related complications.
SUBJECTS, MATERIALS AND METHODS: A cross-sectional study comprising sixty one sickle
cell subjects and thirty healthy haemoglobin-AA control subjects was carried out. Protein C antigen
(quantitative) and activity (qualitative) levels were assayed using enzyme linked immunosorbent
assay and a microtitre plate adjustment of the protac method respectively. Haematological parameters
were measured with the haematology automated analyzer.
RESULTS: The adult SCD subjects had lower values for both PC antigen (68.6 ± 16.0 %; p=0.004)
and activity (49.0 ±13.0 %; p=0.005) when compared to the adult control group (PC antigen = 84.8 ±
18.0 %; PC activity = 60.31±10.37 %). Similarly the paediatric SCD subjects had lower PC antigen
(54.9 ± 14.9 %; p=<0.001) and activity (48.0 ± 13.1 %; p=0.006) levels compared to their control
group (PC antigen = 79.6 ± 16.7 %; PC activity = 58.6 ± 8.0 %). However there was no significant
association between PC levels and SCD complications or vaso-occlusive events in both paediatric and
adult subjects.
CONCLUSION: There is functional PC deficiency in SCD patients. This supports a hypercoagulable
state in the patients. However there was no significant association between PC levels and SCD
complications or vaso-occlusive events. Thus protein C level in SCD patients may not be a good
prognostic marker for disease severity.

A STUDY OF THE CO-RELATIONSHIP BETWEEN PROTEIN C LEVELS AND COMPLICATIONS OF SICKLE CELL DISEASE IN UNIVERSITY OF BENIN TEACHING HOSPITAL