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.
D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications*; D001284:Atrophy; D005260:Female; D006450:Hemoglobin SC Disease / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D008297:Male; D011877:Radionuclide Imaging; D013154:Spleen / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology; D013159:Splenic Infarction / Q000209:etiology*; D013163:Splenomegaly / Q000209:etiology