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Splenic arteries and veins in pediatric sickle cell disease
J P de Chadarévian1, F S Balarezo, M Heggere
1Department of Pathology and Laboratory Medicine, MCP Hahnemann University School of Medicine and St. Christopher's Hospital for Children, Erie Avenue at Front Street, Philadelphia, PA 19134, USA.
Insights
Large vessel lesions in the spleen are common in sickle cell disease patients, affecting arteries and veins. These vascular anomalies, including intimal proliferation and T-cell infiltration, are prevalent even in young individuals.
Area of Science:
- Vascular Biology
- Hematology
- Pathology
Background:
- Sickle cell disease (SCD) is a genetic blood disorder characterized by abnormal hemoglobin.
- Vascular complications are a major cause of morbidity and mortality in SCD patients.
- The presence and nature of large vessel lesions outside the central nervous system in pediatric SCD are not well-characterized.
Purpose of the Study:
- To investigate the existence and prevalence of large vessel lesions in the spleen of young patients with sickle cell disease.
- To characterize the specific types of vascular anomalies present in affected spleens.
Main Methods:
- Histopathological examination of 17 spleens from SCD patients undergoing resection due to sequestration or infarction.
- Comparison with 41 spleens from control individuals.
- Analysis of vascular structures, including arteries and veins, for specific lesions.
Main Results:
- All spleens from SCD patients exhibited anomalies in arteries and veins.
- Consistent lesions included intimal proliferation, internal elastic lamina reduplication in arteries, and subendothelial T-cell infiltration in veins.
- Endotheliitis was observed in a subset of non-SCD controls, but typically milder than in SCD patients.
Conclusions:
- Large vessel lesions are a consistent finding in the spleens of young sickle cell disease patients.
- Specific vascular pathologies, such as intimal proliferation and T-cell infiltration, are characteristic.
- These findings highlight significant vascular involvement outside the CNS in pediatric SCD.
Abstract:
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.
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