Splenic arteries and veins in pediatric sickle cell disease

The goal of this study was to verify the existence and prevalence of large vessel lesions outside the central nervous system in young patients with si ckle cell disease. Thus, 17 spleens resected because of episodes of sequest ration or infarction and 41 controls were studied. Anomalies of arteries an d veins were detected in all spleens from sickle cell disease patients, but no definite correlation with age, sex, type of sickle hemoglobin, or frequ ency of sequestration episodes could be established. The most consistent le sions were intimal proliferation affecting large arteries and veins, redupl ication of the internal elastic lamina of large arteries, and a lesion not previously documented in this condition, that of subendothelial infiltratio n of the large veins by activated T cells. Endotheliitis showing some simil arity with the one seen in sickle cell disease spleens was noted in 5 of 41 spleens of patients who did not suffer from sickle cell disease. However, when present it was usually mild. Very limited damage to the arterial elast ica was noted in only I of the 41 controls. Minimal endothelial proliferati on was seen in 2 of 41 controls.