Hemolytic jaundice
A jaundice type from excessive red blood cell destruction.
Hemolytic jaundice, also called prehepatic jaundice, occurs when red blood cells are destroyed too rapidly (hemolysis), producing more bilirubin than the liver can process. The liver itself is not at fault unless the patient also has liver disease or damage. It is one of the three main types of jaundice, and its most visible sign is yellowing of the skin and the whites of the eyes.
**Signs and symptoms**
Beyond the yellow discoloration, other symptoms depend on what is causing the hemolysis. For instance, if the cause is sickle cell disease, acute episodes may include severe pain or acute chest syndrome, while long-term complications can involve nerve problems or lung issues. In all cases, lab tests show a rise in unconjugated (indirect) bilirubin. Serum bilirubin levels rarely go above 4 mg/dL unless the patient also has liver disease.
**Causes**
The underlying causes are all disorders that trigger hemolysis.
- **Sickle cell disease**: A gene mutation leads to abnormal hemoglobin, causing chronic hemolytic anemia. Lab findings include high unconjugated bilirubin, high lactate dehydrogenase, and low haptoglobin. - **Thrombotic thrombocytopenic purpura (TTP)**: Low activity of the ADAMTS13 enzyme causes tiny blood clots and severe microangiopathic hemolytic anemia. Labs show very high lactate dehydrogenase, negative Coombs test, and no anti-RBC antibodies. Because hemolysis is intravascular, urine may appear dark from hemoglobin. - **Autoimmune hemolytic anemia (AIHA)**: The body’s own antibodies attack red blood cells, leading to extravascular hemolysis. Labs show high lactate dehydrogenase, low haptoglobin, and a positive Coombs test. About one-third of patients have jaundice or dark urine; most symptoms relate to anemia.
Less common causes include drug-induced hemolysis, thalassemia minor, and congenital dyserythropoietic anemias. Rare causes—such as Bartonella infection, transfusion reactions, or other microangiopathic hemolytic anemias—should be considered if specific symptoms appear.
**Pathophysiology**
Bilirubin overproduction happens through two main routes:
- **Intravascular hemolysis**: Red blood cells break apart inside blood vessels. Hemoglobin binds to haptoglobin, and these complexes are taken up by the liver and spleen.
- Field
- Medicine (Hepatology, Hematology)
- Known for
- Jaundice caused by excessive red blood cell destruction
- Symptoms
- Yellowing of sclera and skin; additional symptoms depend on underlying hemolytic cause
- Common causes
- Sickle cell disease, hereditary spherocytosis, thrombotic thrombocytopenic purpura, autoimmune hemolytic anemia, drug toxicity, thalassemia minor, congenital dyserythropoietic anemias
- Diagnosis
- Visual assessment of skin and sclera; laboratory tests to determine cause
- Treatment
- Cause-specific; intense phototherapy and exchange transfusion for bilirubin excretion
Lore & Background
Causes include sickle cell disease, thrombotic thrombocytopenic purpura, autoimmune hemolytic anemia, drug toxicity, thalassemia minor, and congenital dyserythropoietic anemias. Diagnosis relies on visual assessment and laboratory tests such as urine urobilinogen, complete blood count, serum bilirubin fractionation, and liver enzyme analysis. Treatment is specific to the underlying cause, with phototherapy and exchange transfusion used to help excrete accumulated bilirubin.
Reader's Guide
Hemolytic jaundice is significant as one of the three main categories of jaundice, distinguished by its origin in hemolysis rather than liver or bile duct dysfunction. Its recognition is critical because complications such as hyperbilirubinemia and chronic bilirubin encephalopathy can be deadly without proper treatment. The condition highlights the importance of differentiating prehepatic from hepatic and posthepatic jaundice, as management depends on the underlying cause. The use of visual assessment tools like the Kramer's scale and the Jaundice Eye Colour Index aids in quantification, while laboratory tests confirm the etiology. Understanding the pathophysiology—both intravascular and extravascular hemolysis—explains the predominance of unconjugated bilirubin and the absence of bilirubinuria. The legacy of this classification lies in guiding clinicians to identify hemolytic disorders early, preventing severe outcomes through cause-specific interventions such as exchange transfusion in neonates.
Did You Know?
- Serum bilirubin concentration rarely exceeds 4 mg/dL in hemolytic jaundice unless the patient has concurrent liver disease.
- The Jaundice Eye Colour Index (JECI) uses digital photography of the sclera, where a JECI of 0.1 indicates intense yellow colour.
- In hemolytic jaundice, conjugated bilirubin accounts for less than 15% of total serum bilirubin.
- Complications include hyperbilirubinemia and chronic bilirubin encephalopathy, which may be deadly without proper treatment.
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