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.
Types of Hemolytic Anemia
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 anemia
- Thalassemia
- Red Cell Membrane Disorders
- 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
Hemolytic Anemia Treatment
Hemolytic anemia treatments vary depending on the type and cause of the disease and may include:
- Steroid therapy or intravenous immune globulin 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)
Hemolytic Anemia Treatment
Children and young adults with hemolytic anemia are treated through our Blood Disorders Center.
Evans syndrome is an autoimmune disorder in which a person’s own antibodies attack red blood cells (RBC), which deliver oxygen to body tissues, platelets (which help blood clot), and/or neutrophils (a type of white blood cell that helps fight infections). The disorder resembles a combination of autoimmune hemolytic anemia (AIHA; the premature destruction of RBC), thrombocytopenia (too few platelets), and/or neutropenia (too few neutrophils). These may occur simultaneously or one may follow the other.
There is no known genetic cause of Evans syndrome, and occurrences in families are rare. It is considered a chronic disease, with periods of remission and exacerbation. Thrombocytopenia recurs in about 60 percent of patients, while autoimmune hemolytic anemia (AIHA) recurs in about 30 percent.
Evans Syndrome Treatment
Children with Evans syndrome are treated through our Blood Disorders Center. Continue reading to learn more about Evans syndrome or visit the Blood Disorders Center homepage to learn about our expertise.
Symptoms & Diagnosis
The symptoms of Evans syndrome may be similar in nature to leukemia and lymphoma, so those illnesses must be ruled out before a diagnosis is made. If a child has a low RBC count, symptoms may include:
- Jaundice
- Dark Brown Urine
- Pale Skin
- Weakness
- Fatigue
- Shortness of breath
If a child has a low platelet count, symptoms may include:
- Increased bruising
- Petechia – tiny red dots under the skin that are a result of very small bleeds into the skin
- Increased bleeding symptoms, such as a bloody nose or heavy menses
If a child has a low neutrophil count, symptoms may include:
- Fevers
- Mouth sores
- Increased bacterial infections
Doctors diagnose Evans syndrome with a variety of blood tests, including:
- Complete blood count
- Coombs test – a blood test that identifies the cause of anemia
- Reticulocyte count – a blood test that measures how fast new red blood cells called reticulocytes are made by bone marrow and released into the blood
Other tests may include bone marrow aspiration and biopsy and flow cytometry, a technique for identifying and sorting cells by staining with a fluorescent dye. After all tests are completed, doctors will be able to outline the best treatment options.
Treatment & Care
The first-line treatments for Evan’s syndrome are corticosteroid, such as prednisone, and intravenous immunoglobulin, which help to control acute episodes. Most children respond to this treatment, but relapses frequently occur. Other treatments include:
- Immunosuppressive drugs such as 6-mercaptopurine and mycophenolate mofetil
- Removal of the spleen to improve blood counts, which may be short-lived
- Rituximab – a monoclonal antibody
- Stem cell transplant – for severe, difficult-to-treat cases and the best chance for a long-term cure
Long-term OutlookFor children with Evans syndrome, recurrences of thrombocytopenia, anemia, and/or neutropenia are common. Evans Syndrome can sometimes remain well controlled on long-term immunosuppressant therapy and occasionally patients have spontaneous complete resolution of the condition.
