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 sickle cell disease. Thus, 17 spleens resected because of episodes of sequestration or infarction and 41 controls were studied. Anomalies of arteries and veins were detected in all spleens from sickle cell disease patients, but no definite correlation with age, sex, type of sickle hemoglobin, or frequency of sequestration episodes could be established. The most consistent lesions were intimal proliferation affecting large arteries and veins, reduplication of the internal elastic lamina of large arteries, and a lesion not previously documented in this condition, that of subendothelial infiltration of the large veins by activated T cells. Endotheliitis showing some similarity 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 elastica was noted in only 1 of the 41 controls. Minimal endothelial proliferation was seen in 2 of 41 controls.