Types of hemolysis
Hemolysis is divided by location and cause. Intracellular occurs in the spleen, where macrophages capture altered red blood cells; this form is accompanied by an enlarged spleen and moderate jaundice. Intravascular hemolysis means the destruction of cells directly in the bloodstream: free hemoglobin enters the plasma, binds to haptoglobin, and when it is depleted, it is excreted by the kidneys, turning the urine dark. For this reason, congenital forms with a defect in the membrane, enzyme or hemoglobin itself are distinguished and acquired forms, among which autoimmune ones are most common.
- Intracellular hemolysis in the spleen
- Intravascular hemolysis with hemoglobin in plasma and urine
- Hereditary spherocytosis and membrane defects
- Red blood cell enzyme deficiency
- Thalassemias and sickle cell disease
- Autoimmune forms with warm or cold antibodies
Reasons and provocateurs
Autoimmune hemolytic anemia can be independent or accompany systemic diseases, lymphoproliferative diseases and infections. Some drugs can trigger immune destruction of red blood cells, so the doctor always checks the list of medications taken. With a hereditary deficiency of certain enzymes, an attack is provoked by an infection, certain medications and certain foods, such as faba beans. Mechanical failure occurs in artificial heart valves and in some small vessel conditions. In newborns, the cause is incompatibility between the blood of mother and child.
- Autoimmune diseases and lymphomas
- Viral and bacterial infections
- Selected medications
- Hereditary membrane and enzyme defects
- Mechanical damage to the prosthetic valve
- Incompatibility of maternal and fetal blood
- Transfusion of incompatible blood
Symptoms
The picture consists of manifestations of anemia and signs of hemolysis itself. Common complaints are weakness, dizziness, shortness of breath, palpitations, decreased exercise tolerance. The specific part is yellowness of the skin and sclera without itching, dark urine, enlarged spleen and a feeling of heaviness in the left hypochondrium. In chronic cases, gallstones often form in the gallbladder due to excess bilirubin. With acute massive hemolysis, the condition worsens quickly: chills, fever, lower back pain, severe weakness appear, and this situation requires emergency help.
- Weakness, dizziness, shortness of breath
- Yellowness of the skin and whites of the eyes
- Darkening of urine
- Enlarged spleen
- Gallbladder stones with a long course
- Chills, fever and lower back pain in acute crisis
Diagnostics
First, the fact of hemolysis is confirmed: in a general blood test, hemoglobin is reduced, reticulocytes are increased, in biochemistry indirect bilirubin and LDH increase, and haptoglobin, on the contrary, decreases. Then they look for the reason. The direct Coombs test determines whether there are antibodies on red blood cells and separates autoimmune forms from others. A blood smear shows characteristic changes in cell shape. According to indications, hemoglobin electrophoresis is performed if thalassemia is suspected, enzyme and osmotic resistance studies, ultrasound of the spleen and gallbladder.
- General blood test and smear
- Reticulocytes
- Indirect bilirubin, LDH, haptoglobin
- Direct Coombs test
- Hemoglobin electrophoresis according to indications
- Ultrasound of the spleen and gallbladder
- Screening for infections and autoimmune diseases
Treatment and observation
Treatment depends entirely on the cause, and there is no universal treatment. In the autoimmune form, glucocorticoids serve as the basis; if the effect is insufficient, immunosuppressive or anti-B-cell drugs are added. If hemolysis is caused by the drug, it is discontinued. In hereditary forms, it is important to prevent provoking factors and take folic acid, and in severe cases, removal of the spleen is discussed with mandatory preliminary vaccination. Red blood cell transfusion is carried out according to strict indications. Iron supplements without a confirmed deficiency are not prescribed for hemolysis.
- Glucocorticoids for autoimmune form
- Immunosuppressive and anti-B cell drugs
- Discontinuation of the culprit drug
- Folic acid for chronic hemolysis
- Splenectomy according to indications after vaccination
- Red blood cell transfusion for severe anemia
- Do not take iron without a proven deficiency