Hemoglobin S‐C disease revisited: Clinical study of 106 adults

We describe the clinical features of S‐C hemoglobin disease in 106 adults seen during the years 1972–2000 and followed for a mean period of 6.8 years (range 1–27 years). The median age of the patients was 50 years. Common clinical features were pain crisis (65%), avascular necrosis of the hip (23%), proliferative sickle retinopathy (34%), and splenic infarction/splenic sequestration syndrome (19%). Acute splenic sequestration crisis occurred in 10 patients and was the presenting feature in two. Obesity (19.8%), essential hypertension (20.7%), and type‐2 diabetes mellitus (10.3%) were common. The frequent occurrence of these co‐morbidities among patients with hemoglobin S‐C disease has not been reported previously. Am. J. Hematol. 68:298–300, 2001. © 2001 Wiley‐Liss, Inc.