Heinz body formation in red cell membrane disorders: its acceleration in membrane lipid abnormalities.
Abnormal Heinz body formation in the presence of acetylphenyl hydrazine was observed in some patients with red cell membrane abnormalities, such as hereditary red cell membrane high phosphatidyl choline haemolytic anaemia, congenital haemolytic anaemias of unknown origin, acquired hyperlipidaemia and paroxysmal nocturnal haemoglobinuria. No abnormality of haemoglobin composition or of oxidation-reduction activities was noted in 66 patients studied. No abnormal Heinz body formation was seen in hereditary spherocytosis, hereditary elliptocytosis or hereditary stomatocytosis with normal membrane lipids, but increased Heinz body formation was observed in some patients with red cell membrane lipid abnormalities. The extent of abnormal Heinz body formation inversely correlated with a decreased molar ratio of free cholesterol to phosphatidyl choline in these red cells. Heinz body formation, therefore, may be abnormal in some red cell membrane disorders, especially when membrane lipid abnormalities exist.
D000743:Anemia, Hemolytic / Q000097:blood; D002784:Cholesterol / Q000097:blood; D004612:Elliptocytosis, Hereditary / Q000097:blood; D004910:Erythrocyte Membrane / Q000378:metabolism*; D004913:Erythrocytes, Abnormal / Q000378:metabolism*; D006366:Heinz Bodies; D006446:Hemoglobin E; D006449:Hemoglobin M; D006801:Humans; D008563:Membrane Lipids / Q000032:analysis / Q000378:metabolism*; D010713:Phosphatidylcholines / Q000097:blood; D010743:Phospholipids / Q000097:blood; D012964:Sodium / Q000097:blood; D013103:Spherocytosis, Hereditary / Q000097:blood; D013789:Thalassemia / Q000097:blood
D008563:Membrane Lipids; D010713:Phosphatidylcholines; D010743:Phospholipids; D006446:Hemoglobin E; D006449:Hemoglobin M; D002784:Cholesterol; D012964:Sodium