Intravascular hemolysis is manifested by which of the following?

• A. Anemia, Splenomegaly, Jaundice
• B. Anemia only
• C. Anemia, hemoglobinemia, hemoglobinuria, hemosiderinuria, and jaundice
• D. Hyperbilirubinemia with leukocytosis

Answer: (B)

Intravascular hemolysis means red blood cells are destroyed inside the circulating blood, releasing free hemoglobin into the plasma. This produces several characteristic signs. Anemia results from the loss of red cells. Free hemoglobin binds to haptoglobin in the plasma; once haptoglobin is saturated, hemoglobin appears in the blood (hemoglobinemia) and is filtered by the kidneys, causing hemoglobin in the urine (hemoglobinuria). Prolonged or ongoing intravascular destruction can lead to hemosiderin-containing urine (hemosiderinuria) as iron from the breakdown is excreted. The heme breakdown also raises bilirubin, usually increasing unconjugated bilirubin and producing jaundice. Splenomegaly is more typical of extravascular hemolysis, where cells are destroyed in the spleen. Hyperbilirubinemia with leukocytosis isn’t a characteristic pattern of intravascular hemolysis. Therefore, the finding that includes anemia plus hemoglobinemia, hemoglobinuria, hemosiderinuria, and jaundice best fits intravascular hemolysis.