Splenic infarction, splenic sequestration, and functional hyposplenism in hemoglobin S-C disease

Splenic atrophy or evidence of hyposplenism occurs in as many as one third of all patients with S-C hemoglobinopathy. Yet there are few reports in the literature of clinically apparent splenic infarction in this disease. We describe four instances of acute splenic infarction in three patients with hemoglobin S-C disease which illustrate a wide spectrum of clinical manifestations and severity. Of particular interest were the observations of coincident occurrences of splenic sequestration and functional hyposplenism with splenic infarction, suggesting a close pathophysiological relationship among these syndromes.