Traditional laboratory measures of cardiovascular risk in hereditary spherocytosis

Background: Individuals who have undergone splenectomy may be at an increased risk of arterial and venous thrombosis. We sought to determine if splenectomy affects surrogate laboratory measures of cardiovascular risk in persons with hereditary spherocytosis (HS). Procedure: We conducted a prospective cross-sectional study of 21 children and 36 adults with HS. Fasting blood samples were collected for complete blood count and plasma lipid panel, homocysteine, lipoprotein (a), C-reactive protein, and fibrinogen. The variables were compared between the groups with and without prior splenectomy by Mann-Whitney tests. Results: Subjects with prior splenectomy had higher hemoglobin, white blood cell and platelet counts and lower reticulocyte counts and total serum bilirubin concentrations (P < 0.001). Subjects not having had splenectomy had lower than normal levels of total and LDL-cholesterol (LDL-C). Total and LDL-C values were significantly higher, as were fibrinogen and homocysteine concentrations, in the post-splenectomy subjects than in individuals with intact spleen. Conclusion: Various lipid levels and other measures of cardiovascular risk are affected by splenectomy in persons with HS. Further investigations are indicated to more clearly define the balance of the potential benefits of hemolysis and anemia versus the deleterious effects of splenectomy in HS. Pediatr Blood Cancer.