Complement factor H levels in steady state sickle cell anaemia

Objective: The red cell membrane of sickle cell anaemia is vulnerable to attack from the alternative complement pathway. The activation of the alternative complement pathway is initiated by externalization of phosphatidylserine on red cell membrane. Serum and cell bound regulators normally prevent amplification of the cascade. However, red blood cells in sickle cell anaemia appear to be exposed and the cell lysing membrane attack complex is ubiquitous on irreversible sickle red blood cells. It is possible that there are deficiencies (either functional or quantitative) of complement regulators. In this study the quantitative defects of the most abundant serum phase regulator, complement factor H in sickle cell anaemia was investigated.