Hereditary Heinz‐Body Anaemia

Three-dimensional structure of a hemichrome hemoglobin fromCaudina arenicola

“…Invertebrate and vertebrate hemoglobins can form hemichrome hemoglobins by a similar mechanism (Spagnuolo, Rinelli, Coletta, Chiancone & Ascoli, 1987) using reducing agents such as nitrite (Spagnuolo et al, 1988). Hemichrome formation in human red blood cells can be the result of abnormalities in the ratio of ot and/3 chains or in the enzyme systems that protect hemoglobins against oxidation (Dacie et al, 1964). Studies on human hemoglobin tetramers as well as the individual chains have shown that the combination of unlike subunits can prevent hemichrome formation (Rachmilewitz, Peisach & Blumberg, 1971).…”

“…Hemoglobin H (otthalassemia) is a disease characterized by hemichrome formation related to an over abundance of /3-chains (Dickerson & Geis, 1983). The/3-chains assemble into/34 molecules that have high oxygen affinity, do not bind oxygen cooperatively (Kurtz, Rollema & Baurer, 1981) and form hemichromes, causing the hemoglobin molecule to precipitate out of solution and form inclusion bodies in the cell, referred to as Heinz bodies (Dacie et al, 1964). High-resolution X-ray crystallographic structures of human /34 have been determined in the carbonmonoxy and deoxy states (Borgstahl, Rogers & Arnone, 1994a,b) that draw interesting conclusions about the stability of the 134 molecule and the conversion of the human (ot/3)2 heterotetramer from the deoxy to oxy states, but there is no evidence of hemichrome Fe atom coordination in the deoxy structure.…”

Congenital Heinz Body Haemolytic Anaemia Due to Haemoglobin Perth in a Nama Child Seemingly Aggravated by the High Nitrate Content of the Water Supply

“…HbA2 determinations were carried out by elution of the component separated on cellulose acetate and HbF estimation made as described by Betke ei al. [6], The heat stability test was carried out according to the methods of Dacie et al [7] and Carrell and Kay [8].…”

Hemoglobin Hasharon in a Premature Infant with Hemolytic Anemia

“…Heinz body preparations were made with 0.5% methyl violet in normal saline. Thermolabile hemoglobin was tested by the method of Dacie et al (5) in 0.1 M Tris buffer, pH 7.4, at 50'. Glucose 6-phosphate dehydrogenase activity was determined by the method of Motulsky (19).…”