Table 2

Hypothetical risk of vascular complications based on the presence or absence of intravascular hemolysis and splenic function

Pathophysiologic stateExampleRisk of vascular complications
Neither hemolysis nor splenectomy Normal person Baseline* 
Hemolysis with intact spleen Hereditary spherocytosis Glucose-6-phosphate dehydrogenase deficiency β-thalassemia trait Other chronic hemolytic anemia Baseline* or decreased 
Splenectomy without hemolysis Hereditary spherocytosis Immune thrombocytopenic purpura Trauma Increased or baseline* 
Splenectomy and ongoing hemolysis Thalassemia intermedia Hgb E/β-thalassemia Sickle cell anemia§ Hereditary stomatocytosis Greatly increased 
Pathophysiologic stateExampleRisk of vascular complications
Neither hemolysis nor splenectomy Normal person Baseline* 
Hemolysis with intact spleen Hereditary spherocytosis Glucose-6-phosphate dehydrogenase deficiency β-thalassemia trait Other chronic hemolytic anemia Baseline* or decreased 
Splenectomy without hemolysis Hereditary spherocytosis Immune thrombocytopenic purpura Trauma Increased or baseline* 
Splenectomy and ongoing hemolysis Thalassemia intermedia Hgb E/β-thalassemia Sickle cell anemia§ Hereditary stomatocytosis Greatly increased 
*

Dependent on the person's unique genetic and environmental risk factors.

Very mild hemolysis may be present in some persons.

Especially intravascular hemolysis.

§

Nearly all patients lack functional spleens.