Hemolytic Anemia

Hemolytic anemia is a sub-type of anemia, a common blood disorder that occurs when the body has fewer red blood cells than normal. In hemolytic anemias, the low red blood cell count is caused by the destruction — rather than the underproduction — of red blood cells. It occurs when red blood cells are destroyed faster than the bone marrow can make them. Red blood cells carry oxygen from the lungs to all parts of the body.

There are two types of hemolytic anemia:

• Inherited hemolytic anemia (also called intrinsic hemolytic anemia) is caused by a defect in the red blood cells themselves and result when one or more genes that control red blood cell production don’t function properly. With these conditions, red blood cells are destroyed earlier than normal.
• Acquired hemolytic anemia (also called extrinsic hemolytic anemia) is caused by factors outside the red blood cell, such as antibodies from an autoimmune disorder, burns, or medications. In these conditions, red blood cells are usually healthy when they are produced by the bone marrow, but later they are destroyed directly in the bloodstream or get prematurely trapped and recycled in the spleen.

Types of inherited hemolytic anemia include:

• Sickle cell disease
• Thalassemia
• Ted cell membrane disorders, such as hereditary spherocytosis, hereditary elliptocytosis and hereditary pyropoikliocytosis, hereditary stomatocytosis and hereditary xeocytosis
• Pyruvate kinase deficiency (PKD)
• Glucose-6-phosphate dehydrogenase (G6PD) deficiency

Types of acquired hemolytic anemia include:

• Immune hemolytic anemia
• Autoimmunehemolytic anemia (AIHA)
• Alloimmune hemolytic anemia
• Drug-induced hemolytic anemia
• Mechanical hemolytic anemias
• Paroxysmal nocturnal hemoglobinuria (PNH)
• Malaria, babesiosis, and other infectious anemias

It is important to understand some symptoms of anemias may resemble those of other more common medical problems or other blood disorders. Because some of these symptoms can also point to other conditions, and because anemia itself can be a symptom of another medical problem, it’s important to have your child evaluated by a qualified medical professional for an accurate diagnosis and prompt treatment.

• Pale skin
• Jaundice, or yellowing of the skin and eyes
• Dark-colored urine
• Fever
• Weakness
• Fast heart beat

Hemolytic anemia may be caused by several different factors that may be difficult to isolate. It is important to talk to your child's doctor about possible underlying conditions that may cause hemolytic anemia. Hemolytic anemia can be categorized into two types — inherited and acquired.

Typically a physical exam and blood tests are used to diagnose hemolytic anemia. Additional procedures may include a urine, bone marrow, or genetic test. People who are diagnosed with mild hemolytic anemia may not need treatment at all. For others, hemolytic anemia can often be treated or controlled.

Hemolytic anemia treatments vary depending on the type and cause of the disease and may include:

• Steroid therapy to decrease the immune system attack on the red blood cells
• Red blood cell transfusion, may be used when anemia is severe or therapy is ineffective

If a child has severe hemolytic anemia, hospitalization may be needed for the following:

• Exchange transfusion to replace damaged blood with fresh blood
• Surgery to remove the spleen (usually reserved for children who don’t respond to other therapies)

Children and young adults with hemolytic anemia are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through our Blood Disorders Center. Our program brings together world-renown pediatric hematology specialists and support staff from across Dana-Farber/Boston Children’s, including pediatric hematologist/oncologists, hematopathologists, hematology nurse practitioners, social workers and designated hematology patient coordinators. For many appointments and certain procedures, your child can also receive care at one of Boston Children's satellite offices.