Volume 1

The haemolytic anaemias, congenital and acquired / by J.V. Dacie.

  • Dacie, John V. (John Vivian), Sir.
Date:
1960-1967
Catalogue details

Licence: Attribution-NonCommercial 4.0 International (CC BY-NC 4.0)

Credit: The haemolytic anaemias, congenital and acquired / by J.V. Dacie. Source: Wellcome Collection.

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    Allison and ap Rees (1957) have also published interesting observations on the relationship between the plasma haptoglobins, renal threshold and haemoglobinuria. Also using starch-gel electrophoresis, they confirmed that the great majority of subjects fall into one of three groups: 1—1, with only one haptoglobin migrating in the fast a 2 position; 2-2, with three haptoglobins migrating as three distinct bands in the ccp region, and 1-2, with four haptoglobins, three migrating slightly faster than those of group 2-2, plus one which is apparently the same as that of group 1—1. Allison and ap Rees also reported that umbilical- cord blood was distinctive in not containing demonstrable haptoglobins and that a small percentage of older children and adults have sera either lacking haptoglobins or, at the most, containing a single component giving a band in the region. Allison and ap Rees also added oxyhsemoglobin to serum in vitro. When more than 100-135 mg. per 100 ml. were added free oxyhsemoglobin could be demonstrated, migrating as a band distinct from the haemoglobin-haptoglobin complexes, as well as methaemalbu- min, which migrated in the albumin region. They, too, concluded that the renal threshold for haemoglobin depends primarily on the haptoglobin level of the plasma. Several patients suffering from intravascular haemolysis were studied. In four patients with paroxysmal nocturnal haemoglobinuria and in two patients with paroxysmal cold haemoglobinuria haptoglobins were absent or present in quantities too small to be detected, and in one case of march haemoglobinuria the haptoglobin concentration was less than half the normal value. In one possible case of paroxysmal nocturnal haemoglobinuria, examined at least 8 years after apparent complete recovery, the haptoglobins were normal, as they were also in a patient with paroxysmal cold haemoglobinuria, who had a single attack several years previously. Allison and ap Rees pointed out that the low haptoglobin levels of these patients could be the result of either diminished formation, for genetic or other reasons, or rapid removal from the blood stream as the result of the continuing intravascular haemolysis. The second hypothesis seems the more likely on the available evidence. Aber, Neale and Northam (1957) and Neale, Aber and Northam (1958) studied, using simple paper electrophoresis, the binding of haemoglobin by serum in vitro with particular reference to megaloblastic anaemia and haemolytic anaemia. They also have concluded that the serum proteins capable of binding haemoglobin are lost from the circulation in the process of clearing the plasma of haemoglobin. Using paper electrophoresis, Aber, Neale and Northam have found that a normal serum will bind haemoglobin to a 2 globulin, /^-globulin and albumin in that order, and that following a haemolytic episode the ^-globulin binding-component returns first and then the a 2 globulin. They concluded that the behaviour of serum in respect of binding haemoglobin, when haemoglobin is added at a concentration of 30 mg. per 100 ml., provides important evidence as to whether intravascular haemolysis is occurring or has recently taken place. Brus and Lewis (1959) have more recently reported on the results of a survey of a large number of patients with various types of haemolytic anaemia. Measurement of erythrocyte survival by the radiochromium method and the demonstration of haptoglobins using cellulose-acetate