Delayed adverse vascular events after splenectomy in hereditary spherocytosis

Abstract

To cite this article: Schilling RF, Gangnon RE, Traver MI. Delayed adverse vascular events after splenectomy in hereditary spherocytosis. J Thromb Haemost 2008; 6: 1289-95.Summary. Background: It is probable that the variety and frequency of delayed adverse vascular events after splenectomy are underappreciated. Splenectomy is performed for a wide variety of conditions, and delayed postsplenectomy hazards are not often studied. Objective: To estimate the relative risk of adverse vascular events in members of hereditary spherocytosis families who have or have not had a splenectomy. Methods: Members of families in which hereditary spherocytosis exists were systematically questioned about adverse vascular events. Results: The cumulative incidence of arterial and venous events at age 70 years was greater in persons who had undergone a splenectomy for spherocytosis (arterial, 22% females, 32% males; venous, 20% females, 19% males) than in affected persons who did not undergo splenectomy (arterial, 3% females, 2% males; venous, 6% females, 4% males) or non-affected family members (arterial, 10% females, 17% males; venous, 4% females, 12% males). Affected subjects who undergo splenectomy are at greatly increased risk of arterial events as compared to affected subjects who do not undergo splenectomy [arterial, hazard ratio (HR) 7.2, 95% confidence interval (CI) 2.8-17.2; venous, HR 3.3, 95% CI 1.1-9.8]. Conclusion: There is a significant, long-lasting, increased risk of adverse arterial and venous thromboembolic events after splenectomy performed for hereditary spherocytosis. A review of the literature indicates that this is also true when splenectomy is performed for several other indications.

Patients After Splenectomy: Old Risks and New Perspectives

“…According to Schilling, splenectomized patients suffering from hereditary spherocytosis (HS) are seven times more likely to suffer an arterial event and three times more likely to undergo a venous event when comparing them to HS patients who had their spleen preserved (22).…”

A novel hemoglobin-binding peptide reduces cell-free hemoglobin in murine hemolytic anemia

“…Indeed, intravascular hemolysis has been linked to pulmonary hypertension (19,24,26,45,59), systemic vasculopathies (27,28,50,52,53,60), and cardiovascular complications (11,25,30,39). Although such studies have suggested that CF-Hb likely plays important roles in vascular disease, the actual contribution of CF-Hb to disease pathology has drawn controversy, particularly in sickle cell disease (SCD) (9,18).…”

Long-term follow-up of subtotal splenectomy for hereditary spherocytosis: a single-center study

“…[5][6][7][8] However, spleen removal is associated with loss of important physiological functions: splenectomized patients are exposed to long-term complications including infectious risks (particularly high in children under 6 years of age 9 ) and vascular risks. 10,11 To preserve the spleen function, subtotal splenectomy (STS) has been proposed as an alternative to TS. We and others published reports regarding a series of HS patients who underwent STS and showed that it reduced the hemolytic rate and increased the red cell life span while maintaining efficient splenic phagocytic function.…”