Sickle cell anemia is recognized as a cause of priapism, but other hemolytic anemias and hemoglobinopathies may be associated with priapism as well.
Hemolytic anemias associated with priapism:
(1) sickle cell disease
(2) thalassemia
(3) hereditary spherocytosis
(4) paroxysmal nocturnal hemoglobinopathy
(5) hemoglobinopathies with unstable hemoglobin
(6) glucose-6 phosphatase deficiency
(7) glucose-6 isomerase deficiency
(8) congenital dyserythropoietic anemia
Splenectomy or asplenia may increase the risk of priapism in these patients.
