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 causingÂ factor of anemia. Patients with minimal hemolytic anemia may be asymptomatic for long periodsÂ of timeÂ so hemolysis is discovered accidentallyÂ during routine testing.
During intravascular hemolysis, iron deficiency is caused by elimination of hemoglobin in urineÂ to chronicÂ that worsens anemia and bodyÂ 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 Â hemochromatosis orÂ incorrect iron therapy.Â Elimination of hemoglobin in the urine produces aÂ dark urine,Â that canÂ be presentÂ 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,Â 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 symptomsÂ of the underlying disease.
- Generalized pale conjunctiva, pale nails indicate anemia but are not specific for hemolytic anemia.
- Tachycardia, tachypnea, and hypotension due toÂ anoxia (lack of oxygen)Â and vascular volume decrease,Â usually occur in severe anemia but are not specific for hemolytic anemia.
- Jaundice may occur with due toÂ increased indirect bilirubinÂ 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 isÂ not complicated with liver disease or cholelithiasis.
- Splenomegaly occurs in hemolytic anemia and spherocytosis but is not present in G6PD deficiency. TheÂ presence of splenomegaly suggestsÂ diseases such as chronic lymphocytic leukemia, lymphomas and lupus erythematosus. Right upper quadrant abdominal sensitivity indicates biliary disease. Bleeding andÂ petechiaeÂ with thrombocytopeniaÂ indicate Evans syndrome or thrombotic thrombocytopenic purpura if neurological signs are present.Â Lymphadenopathy with splenomegalyÂ indicates chronic lymphocytic leukemia.
Mortality is usuallyÂ low in cases of hemolytic anemia. Elderly patients and those with cardiovascular riskÂ have anÂ increased risk. Morbidity is dependent on the etiology of hemolysis and the underlying diseaseÂ like sickle cell diseaseÂ or malaria. Tachycardia and dyspnoea occur when the onset and hemolysisÂ 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 PÂ on the erythrocyte surface. Antibodies attach to the erythrocyte surface in cold weather, below 30 degrees. Anemia resolves spontaneously, but inÂ 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 toÂ avoid cardiac stress. The risk of acute hemolysis due to transfusion is increased but the degree of hemolysis is dependent on theÂ infusion rate. Different slow infusions should be administered to prevent rapidÂ destruction of erythrocytes. Iron overload by repeated transfusions for chronic anemia can be treated with chelation therapy. Chelating agents likeÂ deferoxamine and deferiprone are used. Drugs that promote hemolysis should be removed. Folic acid, corticosteroids, iron therapy is indicatedÂ in autoimmune cases.
Penicillin and other agents that cause immune hemolysis andÂ drugs such as drug oxidizing sulfateÂ are contraindicated:
- Penicillin, cephalothin, ampicillin
- Meticilin, quinina, quinidine.
Folic acidÂ is administredÂ becauseÂ active hemolysis can cause megaloblastic anemia and folate consumption. Intravenous immunoglobulin G is used for patients with autoimmune hemolytic anemia, but onlyÂ some patients respond to treatment and response is transient.
Splenectomy (surgical spelin removal)Â may be the first choice in some types of hereditary hemolytic anemia as sperocytosis. In other cases it is recommended when otherÂ medical treatmentsÂ 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 shouldÂ avoided in G6PD deficiency include:
- Acetanilide, furazolidone, isobutyl nitrite
- Nalidixic acid, naphthalene, niridazol.
Hemolytic anemia prognosis depends on underlying disease.