Hemolytic anemia can occur in people of any age. Hereditary disorders are usually evident early in life. Autoimmune hemolytic anemia is more common in people of middle age and elderly persons. Symptoms of hemolytic anemia are diverse and are due to anemia, ongoing treatments and the causing factor of anemia. Patients with minimal hemolytic anemia may be asymptomatic for long periods of time so hemolysis is discovered accidentally during routine testing.
During intravascular hemolysis, iron deficiency is caused by elimination of hemoglobin in urine to chronic that worsens anemia and body weakness. Tachycardia, dyspnea, angina and weakness occur in patients with severe anemia. Cardiac function is sensitive to anoxia (lack of oxygen). Angina and cardiac decompensation in patients with severe anemia occurs if the onset is sudden. Gallstones can cause abdominal pain. Bilirubin stones may develop in patients with persistent hemolytic anemia. Tanned skin color and diabetes result from multiple transfusions hemochromatosis or incorrect iron therapy. Elimination of hemoglobin in the urine produces a dark urine, that can be present in patients with intravascular hemolysis and transfusion from a incompatible blood group. Thrombotic thrombocytopenic purpura patients may experience fever, neurological signs, renal failure, petechiae, and hemolysis due to small vessel occlusion expansion. Foot ulcers can develop in patients with sickle cell disease or hemolysis as a result of decreased red blood cell deformation.
Penicillin, quinina, quinidine, L-dopa and other agents can cause immune hemolysis. Oxidative drugs, infection stress can trigger hemolysis in patients with G6PD deficiency. Fava beans can cause hemolysis in individuals with susceptible Mediterranean G6PD deficiency.
Physical examination in patients with hemolytic anemia shows signs of anemia, destruction of red blood cells, hemolysis and signs and symptoms of the underlying disease.
- Generalized pale conjunctiva, pale nails indicate anemia but are not specific for hemolytic anemia.
- Tachycardia, tachypnea, and hypotension due to anoxia (lack of oxygen) and vascular volume decrease, usually occur in severe anemia but are not specific for hemolytic anemia.
- Jaundice may occur with due to increased indirect bilirubin in hemolysis. The increase is not specific for hemolytic disease and can occur in liver disease, biliary obstruction and congenital liver disease. Bilirubin level is rarely more than 4 mg / dL in hemolysis if it is not complicated with liver disease or cholelithiasis.
- Splenomegaly occurs in hemolytic anemia and spherocytosis but is not present in G6PD deficiency. The presence of splenomegaly suggests diseases such as chronic lymphocytic leukemia, lymphomas and lupus erythematosus. Right upper quadrant abdominal sensitivity indicates biliary disease. Bleeding and petechiae with thrombocytopenia indicate Evans syndrome or thrombotic thrombocytopenic purpura if neurological signs are present. Lymphadenopathy with splenomegaly indicates chronic lymphocytic leukemia.
Mortality is usually low in cases of hemolytic anemia. Elderly patients and those with cardiovascular risk have an increased risk. Morbidity is dependent on the etiology of hemolysis and the underlying disease like sickle cell disease or malaria. Tachycardia and dyspnoea occur when the onset and hemolysis sudden and the anemia is severe. Angina and heart failure occurs in patients with cardiovascular disease and decompensated severe hemolysis. Haemosiderosis, leg ulcers, gallstones and folate deficiency may also occur.
Donath-Landsteiner hemolytic anemia
It is a common form of hemolytic anemia in children, intravascular caused by exposure to cold with abnormal immunoglobulin G reactivity to cold directed against an antigen P on the erythrocyte surface. Antibodies attach to the erythrocyte surface in cold weather, below 30 degrees. Anemia resolves spontaneously, but in severe cases can cause shock heart failure or kidney failure. Classic symptoms of hemolytic anemia include sudden onset of pallor and jaundice accompanied by elimination of hemoglobin in urine. Hemoglobinuria resolves spontaneously. Anemia can be mild, describing fever, chills, back or leg pain, abdominal cramps along with headaches, nausea and vomiting.
Hemolytic Anemia Treatment
Therapy is different for each type of hemolytic anemia. Transfusions are indicated only in patients with severe angina or severe cardiopulmonary compromised status.
Different erythrocyte concentrates will be administred to avoid cardiac stress. The risk of acute hemolysis due to transfusion is increased but the degree of hemolysis is dependent on the infusion rate. Different slow infusions should be administered to prevent rapid destruction of erythrocytes. Iron overload by repeated transfusions for chronic anemia can be treated with chelation therapy. Chelating agents like deferoxamine and deferiprone are used. Drugs that promote hemolysis should be removed. Folic acid, corticosteroids, iron therapy is indicated in autoimmune cases.
Penicillin and other agents that cause immune hemolysis and drugs such as drug oxidizing sulfate are contraindicated:
- Penicillin, cephalothin, ampicillin
- Meticilin, quinina, quinidine.
Folic acid is administred because active hemolysis can cause megaloblastic anemia and folate consumption. Intravenous immunoglobulin G is used for patients with autoimmune hemolytic anemia, but only some patients respond to treatment and response is transient.
Splenectomy (surgical spelin removal) may be the first choice in some types of hereditary hemolytic anemia as sperocytosis. In other cases it is recommended when other medical treatments have failed. Splenectomy is usually not recommended in hemolytic anemia with cold agglutinins. Immunization against encapsulated organisms infections such as Haemophilus influenzae and Streptococcus pneumoniae is indicated.
Fava beans can cause severe hemolysis in some populations with Mediterranean variant of G6PD deficiency.
Drugs that should avoided in G6PD deficiency include:
- Acetanilide, furazolidone, isobutyl nitrite
- Nalidixic acid, naphthalene, niridazol.
Hemolytic anemia prognosis depends on underlying disease.