Homozygous Sickle Cell Disease and Priapism in the Eastern Province of Saudi Arabia

Authors:
  • HKHuxley Knox-Macaulay
    Huxley Knox-Macaulay
Publisher website
Google Scholar
Abstract
Two adult sickle cell homozygotes from the eastern oases of the Kingdom of Saudi Arabia presented with severe persistent priapism. Each patient had a high Hb F of 24%, and their red cells were neither hypochromic nor microcytic. Priapism probably occurs more frequently in homozygous sickle cell disease than reported previously from this region. It would appear that a high fetal haemoglobin alone without hypochromia and microcytosis – features suggestive of co-existing alpha-thalassaemia – does not protect against this agonising vaso-occlusive event.