Brief overview:
- What is hemolytic anemia? Anemia caused by destruction or premature breakdown of red blood cells (erythrocytes).
- Course of disease and prognosis: Course and prognosis depend on the underlying cause.
- Symptoms: Pallor, weakness, circulatory problems up to fainting, headache, abdominal pain, back pain, yellowing of skin and mucous membranes (icterus), enlargement of the spleen (splenomegaly).
- Causes: Congenital or acquired diseases, medications, drugs.
- Treatment: Glucocorticoids (cortisone), immunosuppressants (drugs that lower the immune response), bone marrow transplantation, removal of the spleen, administration of folic acid and iron.
- Prevention: no specific preventive measures are possible.
What is hemolytic anemia?
In hemolytic anemia, this cycle is shortened: the red blood cells are broken down prematurely (on average after about 30 days), and new formation in the bone marrow lags behind. Overall, there are too few red blood cells in the blood and breakdown products of the blood cells are deposited in the body. Typical signs of hemolytic anemia are pallor, fatigue, dizziness, circulatory problems, yellowing of the skin and enlargement of the spleen.
In addition, doctors distinguish whether the cause of hemolysis is in the red blood cells themselves (corpuscular anemia) or outside the blood cells (extracorpuscular anemia).
What is hemolysis?
The regular degradation of old erythrocytes is carried out by so-called phagocytes (macrophages) in the spleen, and to a lesser extent in the liver. They dissolve the envelope of the red blood cells and break them down. Macrophages are found in various tissues; physicians refer to them as a whole as the “reticuloendothelial system”.
In hemolytic anemia, more red blood cells break down than usual, and at the same time the bone marrow lags behind in the formation of new cells. The result is that there are too few erythrocytes in total.
What is anemia?
Anemia (anemia) is when the number of red blood cells (and thus also the red blood pigment hemoglobin) is below the age- and sex-specific reference value.
Course of disease and prognosis
Both the course and prognosis of hemolytic anemias depend on the underlying cause.
In any case, it is important to have regular check-ups. In the case of chronic hemolysis, the physician examines the blood closely.
What are the symptoms of hemolytic anemia?
There are different causes of hemolytic anemia. Which symptoms occur depends on the particular trigger of the disease.
The following symptoms are indicative of hemolytic anemia:
- Pallor
- Fatigue
- Reduced performance
- Low blood pressure (hypotension)
- Dizziness
- Headache
- Ringing in the ears
- Circulatory problems up to fainting
- Heart palpitations
- Breathing difficulties
- Yellowing of the mucous membranes and skin (jaundice): Results from the breakdown of the red blood pigment (hemoglobin) contained in red blood cells. The yellowish color is caused by bilirubin, the breakdown product of hemoglobin.
Possible complications of hemolytic anemia
Hemolytic crisis: A hemolytic crisis occurs when a large number of red blood cells dissolve in a short period of time. Such crises are possible, for example, in favism, sickle cell anemia, and blood transfusions. Signs of a hemolytic crisis are:
- Fever
- Chills
- Weakness
- Circulatory problems up to shock
- Abdominal pain
- Back pain
- Headache
- Red or red-brown urine (when hemoglobin is excreted in the urine)
A hemolytic crisis is a medical emergency. Call 911 at the first sign!
Gallstones: As a result of chronic hemolytic anemia, gallstones form in some patients. They form because increased bilirubin is produced when the red blood pigment (hemoglobin) is broken down. It accumulates in the gallbladder and forms so-called “pigment stones” in some patients.
Iron deficiency: If too many red blood cells are lost, iron deficiency occurs in the long term. This is because iron is a component of the red blood pigment hemoglobin.
Cause and risk factors
Corpuscular hemolytic anemia
- Congenital cell membrane disorder: spherocytic cell anemia (hereditary spherocytosis)
- Acquired cell membrane disorder: paroxysmal nocturnal hemoglobinuria
- Erythrocyte metabolism disorder: favism (glucose-6-phosphate dehydrogenase deficiency)
- Hemoglobinopathy: sickle cell disease, thalassemia
Extracorpuscular hemolytic anemias.
Possible causes are:
- Medications: Some agents such as quinine and mefloquine (antimalarials), penicillin, antibiotics such as metronidazole, psychotropic drugs such as bupropion, or pain relievers (NSAIDs) such as ibuprofen may trigger hemolytic anemia.
- Infections: In some cases, infections cause hemolytic anemia. Common pathogens include Clostridium perfringens, Streptococcus, Meningococcus, Plasmodia, Bartonella, Epstein-Barr virus, and Mycoplasma.
- Mechanical injury to erythrocytes: this is where the red blood cells are damaged and destroyed by mechanical obstructions in the bloodstream (for example, artificial heart valves).
- Poisons (toxins): Poisoning with lead or copper causes increased red blood cell breakdown.
- Drugs: Drugs such as ecstasy or cocaine may trigger hemolytic anemia.
Examination and diagnosis
Medical history
In the initial consultation, the physician inquires about the current symptoms and asks how long they have been present. If hemolytic anemia is suspected based on blood findings, the physician inquires about other abnormalities. These include:
- Family history: are there any cases of hemolytic anemia (such as thalassemia, sickle cell anemia, or favism) in the family?
- Is there a fever or other medical conditions?
- Is the patient taking any medications? If yes, which ones?
Blood test
If there are no current blood results, the physician draws blood from the patient and pays particular attention to the following values:
- Decreased number of red blood cells (erythrocytes) and red blood pigment (hemoglobin)
- Increased number of reticulocytes (reticulocytosis, precursor cells of red blood cells in bone marrow)
- Low haptoglobin (transport protein for the red blood pigment hemoglobin)
- Increased bilirubin (bile pigment, sign of increased hemoglobin breakdown)
- Folic acid or iron deficiency
Blood smear
For a blood smear, the physician spreads a drop of blood on a glass slide and examines the individual blood cells under the microscope for changes. Certain changes in the shape of the red blood cells provide clues to specific diseases that cause hemolytic anemia. For example, in spherocytic anemia, the red cells are spherical instead of flat.
Urine examination
Coombs test
The Coombs test is a blood test for the detection of antibodies against red blood cells. With this, the doctor examines whether antibodies against red blood cells are present in the patient’s blood.
Ultrasound examination
An ultrasound examination of the abdomen will reveal whether the spleen and/or liver are enlarged.
Treatment
Glucocorticoids and immunosuppressants: Glucocorticoids (cortisone) and immunosuppressants (drugs that suppress an excessive reaction of the body’s defense system) help in autoimmune hemolytic anemias.
Avoidance of triggering drugs: If the cause of the hemolytic anemia lies in a particular active ingredient, the physician will change the drug and, if necessary, switch to another, equivalent preparation.
Bone marrow transplantation: A cure for sickle cell anemia and thalassemia is possible with a bone marrow transplant. In this procedure, bone marrow from a healthy donor is transferred to the patient.
Protection from cold: In chronic autoimmune hemolytic anemia of the cold type, the most important measure is to protect affected individuals from cold.
