title: Compensatory mechanisms for the severe anaemia caused by haemoglobin Hammersmith.
abstract: A severely anaemic, but asymptomatic patient, who is a heterozygous carrier of haemoglobin Hammersmith (beta42 (CD1) phenylalanine - Serine), has been studied to elucidate the mechanisms resulting in physiological compensation for the anaemia. Four factors have been investigated: the oxygen affinity of her blood, the cardiac output at rest and during exercise, the blood gas indices, and pulmonary function. It was found that due to the presence of Heinz bodies within the erythrocytes, the level of functional, haemoglobin was considerably less (50 g/l) than that measured by standard methods (87 g/l). In addition a moderate degree of arterial hypoxaemia (arterial oxygen tension = 10.7 kPa (80.4 mmHg) was present which could not be explained on the basis of abnormal pulmonary function. Both of these factors would result in tissue hypoxia, but the finding of consistently normal oxygen tensions ('mixed' venous oxygen tension = 5.4 kPa (40.3 mmHg) in blood obtained from the right atrium, suggested that hypoxia was not present. This was explained by a decreased whole blood oxygen affinity (P50 = 4.6 kPa (34.5 mmHg) at pH 7.4) and an increase in the cardiac index (5.3 L.min.-1m-2). The latter was the result of an increased stroke volume (125 - 135 ml), the heart rate being normal (63/min.). During moderate exercise, further increases at cardiac output were brought about by a change in heart rate alone. It has been calculated that the decrease in whole blood oxygen per se could not account for adequate tissue oxygenation. This is confirmed by the finding of an increased cardiac output in this patient. It is suggested that in any severe haemolytic anaemia, even if the whole blood oxygen affinity is low, cardiac output is probably increased to achieve complete physiological compensation.
mesh_terms: D000328:Adult; D000745:Anemia, Hemolytic, Congenital / Q000503:physiopathology*; D002302:Cardiac Output; D005260:Female; D006366:Heinz Bodies; D006439:Hemodynamics; D006453:Hemoglobinopathies / Q000503:physiopathology*; D006455:Hemoglobins, Abnormal; D006579:Heterozygote; D006801:Humans; D000860:Hypoxia; D010108:Oxyhemoglobins / Q000378:metabolism; D012119:Respiration
chemical_list: D006455:Hemoglobins, Abnormal; D010108:Oxyhemoglobins