Methaemoglobinaemia 



H. BARCROFT, Q. H. GIBSON and D. C. HARRISON 



This article contains some account of work done on methaemoglobin- 

 aemia in Belfast from 1943 to 1946, and includes a partial review 

 of the literature up to the present. The effect of ascorbic acid in 

 idiopathic methaemoglobinaemia is described and discussed as well 

 as the results of investigations of the enzyme systems in the erythro- 

 cytes normally responsible for methaemoglobin reduction. 



The work done in Belfast on methaemoglobinaemia began as the result 

 of a remarkable coincidence arising from some experiments on the 

 effect of ascorbic acid on polycythaemia vera carried out by J. Deeny 1 . 

 These experiments had led Deeny to believe that ascorbic acid was of 

 benefit in this condition and he continued to look out for suitable cases 

 on whom the treatment could be tested. In this way he came across 

 two brothers both of whom were deeply cyanosed and who had 

 abnormally high red cell counts. On treating one of the brothers with 

 large doses of ascorbic acid, he found that in about three weeks the 

 skin colour had gradually changed from a deep slaty-blue almost to 

 normal. As the effect was striking, he brought the brothers to the 

 laboratory in Belfast to supplement his clinical observations with 

 objective evidence of the changes in their blood. There, investigation 

 showed the blood of the brothers to contain methaemoglobin : about 

 40 per cent of the total pigment in the untreated case, and about 8 per 

 cent in the treated one. 



On going into the literature it was found that methaemoglobinaemia 

 is a very rare disease, particularly as a familial condition, and, in fact, 

 no familial cases had been described as occurring in Great Britain. The 

 effect of ascorbic acid, however, had been discovered by C. Lian, 

 P. Frumusan and M. Sassier 2 , and used by them in the treatment of 

 their two cases. Although it was clear from the literature that the 

 normal mechanisms for the reduction of methaemoglobin (MHb) 

 depended on the enzymes in the red cells and probably on the oxidation 

 of glucose and lactate 3 , it was not possible to form a clear picture 

 either of the abnormality leading to the accumulation of MHb in our 

 cases, or of the action of ascorbic acid in improving their condition. 

 Since we had the good fortune to have access to these cases, it seemed 

 worth while to carry out work on some of these problems and attempt 

 to devise a theory fitting the cases into a general picture of methaemo- 

 globinaemia. 



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