How can you treat hemolysis
Qualitative hemoglobin defects (hemoglobinopathies)
Sickle cell disease
Sickle cell disease is the most common hemoglobinopathy worldwide, currently over 1000 patients live in Germany, exclusively migrants from Central and West Africa, the countries of the Eastern Mediterranean, the Middle East, India and Central Asia.
A point mutation at position 6 of the beta-hemoglobin chain (Glu-Val) changes the hemoglobin molecule (HbS) in such a way that, in the oxygen-poor state, the hemoglobin changes from its soluble form to a polymer and changes the shape of the erythrocytes into the shape of a sickle . Sickle cell carriers with only one allele are largely symptom-free, with homozgosity or in combination with a thalassemia, sickle cell disease develops.
Blood smear showing evidence of sickle cells (Figure 2: Blood smear from a patient with sickle cell anemia). Hemoglobin analysis with detection of HbS and, if necessary, further H. molecular genetic analysis.
Signs of illness
The deformation of the erythrocytes leads to a blockage of small and tiny blood vessels in internal organs (liver, spleen, bones, brain, retina, kidneys, lungs, etc.). Sickle cell disease leads to circulatory disorders and even heart attacks. The following complications are common:
- Aplastic crisis with severe anemia following viral infections (Parvovirus B19)
- Infections with bacteria (pneumococci, salmonella)
- Acute thoracic syndrome: accumulation of blood in the vessels of the lungs with chest pain, shortness of breath, fever and lack of oxygen
- Pain crises especially in the back, arms, legs, chest and stomach.
- Inflammation of the bones (osteomyelitis)
- Strokes, cerebral haemorrhage
Hydroxyurea lowers the number and intensity of pain crises in some patients and reduces mortality.
Transfusions should be given as cautiously as possible and only given in the event of sequestration and aplastic crises. An exchange transfusion may be required for severe complications with infarcts or organ failure.
The avoidance or treatment of complications is crucial for the patient's prognosis. Timely antibiotic therapy for infections and prophylactic vaccinations are part of the treatment concept. The guidelines are updated regularly and can be viewed on the Internet at major centers in the USA and Great Britain (e.g. http://www.nhlbi.nih.gov/health/prof/blood/sickle/sc_mngt.pdf, http: // www .sicklecelldisease.org)
The only potentially curative (healing-oriented) treatment so far has been bone marrow or blood stem cell transplantation. Because of the dangers involved, it is only used for severe forms.
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