[Reduced deformability of erythrocytes as a common denominator of hemolytic anemias].
Impairment of red cell deformability (and decrease of survival time) is the common trait of congenital haemolytic anemias with the exception of the enzyme defect glucose-6-phosphate-dehydrogenase (GPDH) deficiency. Causes for increased red cell rigidity may be: spherocytosis (familial hemolytic anemia), instable less fluid Hb (instable hemoglobinopathy), abnormal HbS-formation (sickle cell disease), genetic synthesis defect of Hb chains (homozygous beta-thalassemia), enzyme defects (autosomal recessive pyruvate-kinase deficiency). With GPDH red cell deformability remains unchanged. Splenectomy may be beneficial in anemias with erythrocyte rigidification if clinical condition so requires (repeated transfusions, aplastic and hemolytic crises). Assessment of red cell deformability in vitro saves laborous and exposure to radiation involving testing of cell survival time (e.g. radiochrome test).
D000745:Anemia, Hemolytic, Congenital / Q000097:blood*; D000755:Anemia, Sickle Cell / Q000097:blood; D002869:Chromosome Aberrations / Q000097:blood; D025063:Chromosome Disorders; D004907:Erythrocyte Deformability; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000097:blood*; D006453:Hemoglobinopathies / Q000097:blood; D006801:Humans; D011770:Pyruvate Kinase / Q000172:deficiency; D013103:Spherocytosis, Hereditary / Q000097:blood; D013789:Thalassemia / Q000097:blood
D011770:Pyruvate Kinase
Verminderte Verformbarkeit der Erythrozyten als gemeinsamer Nenner hämolytischer Anämien.