Causes

Hemolytic anemia occurs when the bone marrow is unable to increase production to make up for the premature destruction of red blood cells. If the bone marrow is able to keep up with the early destruction, anemia does not occur (this is sometimes called compensated hemolysis).

There are many types of hemolytic anemia, which are classified by the reason for the premature destruction of red blood cells. The defect may be in the red blood cell itself (intrinsic factors), or outside the red blood cell (extrinsic factors).

Intrinsic factors are often present at birth (hereditary). They include:

• Abnormalities in the proteins that build normal red blood cells
• Differences in the protein inside a red blood cell that carries oxygen (hemoglobin)

Extrinsic factors include:

• Abnormal immune system responses
• Blood clots in small blood vessels
• Certain infections
• Side effects from medications

Types of hemolytic anemia include:

• Hemoglobin SC disease (similar in symptoms to sickle-cell anemia)
• Hemolytic anemia due to G6PD deficiency
• Hereditary elliptocytosis
• Hereditary ovalocytosis
• Hereditary spherocytosis
• Idiopathic autoimmune hemolytic anemia
• Malaria
• Microangiopathic hemolytic anemia (MAHA)
• Non-immune hemolytic anemia caused by chemicals or toxins
• Paroxysmal nocturnal hemoglobinuria (PNH)
• Secondary immune hemolytic anemia
• Sickle-cell anemia
• Thalassemia
• Transfusion of blood from a donor with a different blood type

Symptoms

• Chills
• Dark urine
• Enlarged spleen
• Fatigue
• Fever
• Pale skin color (pallor)
• Rapid heart rate
• Shortness of breath
• Yellow skin color (jaundice)

Exams and Tests

These are tests for red blood cell destruction (hemolysis). Specific tests can identify the types of hemolytic anemia. They are usually performed when hemolysis is suspected or has been determined.

• Absolute reticulocyte count
• Free hemoglobin in the serum or urine
• Hemosiderin in the urine
• Red blood cell count (RBC), hemoglobin, and hematocrit (HCT)
• Serum haptoglobin levels
• Serum indirect bilirubin levels
• Serum LDH
• Urine and fecal urobilinogen

Directly measuring the red cell life span with radioactive tagging techniques shows a shortened life span.

This disease may also affect the following test results, depending on the specific cause:

• AST
• Coombs' test, direct
• Coombs' test, indirect
• Donath-Landsteiner test
• Febrile or cold agglutinins
• Leukocyte alkaline phosphatase
• Peripheral blood smear
• Platelet count
• Protein electrophoresis - serum
• RBC indices
• Serum creatinine
• Serum ferritin
• Serum iron
• Serum potassium level
• Serum uric acid
• TIBC
• White blood count differential

Treatment

Treatment depends on the type and cause of the hemolytic anemia. Folic acid, iron replacement, and corticosteroids may be used. In emergencies, a blood transfusion or removal of the spleen (splenectomy) may be necessary.

Outlook (Prognosis)

The outcome depends on the type and cause of hemolytic anemia.

Possible Complications

The complications depend on the specific type of hemolytic anemia. Severe anemia can cause cardiovascular collapse (failure of the heart and blood pressure, leading to death). Severe anemias can worsen heart disease, lung disease, or cerebrovascular disease.

When to Contact a Medical Professional

Call for an appointment with your health care provider if you develop symptoms of hemolytic anemia.

Prevention

There is no known prevention for hemolytic anemia.

Alternative Names

Anemia - hemolytic

References

Schwartz RS. Autoimmune and intravascular hemolytic anemias. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 164.

Powers A, Silberstein LE. Autoimmune hemolytic anemia. In: Hoffman R, Benz EJ, Shattil SS, et al, eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Elsevier Churchill Livingstone;2008:chap 47.

Schrier SL, Price EA. Extrinsic nonimmune hemolytic anemias. In: Hoffman R, Benz EJ, Shattil SS, et al, eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Elsevier Churchill Livingstone;2008:chap 48.

Update Date: 1/31/2012

Reviewed by: Linda J. Vorvick, MD, Medical Director, MEDEX Northwest Division of Physician Assitant Studies, University of Washington, School of Medicine; and Yi-Bin Chen, MD, Leukemia/Bone Marrow Transplant Program, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.