GLUCOCORTICOID MANAGEMENT OF PAROXYSMAL NOCTURNAL HÆMOGLOBINURIAL

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Abstract
Summary: In some cases of paroxysmal nocturnal hæmoglobinuria (P.N.H.), glucocorticoids will produce a marked decrease in hæmolysis and an improvement in the hæmoglobin level. In these cases, they can be effective both in the management of life‐threatening hæmolytic crises and in long‐term maintenance therapy. In one case their use has averted a heavy requirement for blood transfusions. In view of the difficulty in withdrawing these drugs, their use is recommended only when more conservative measures are unsatisfactory and the patient's state is causing concern. The régime recommended as a trial is a dose of 40 mg. of prednisone per day for at least two weeks, after which the level is gradually lowered to a maintenance level which has varied between 5 and 12·5 mg. per day for these patients. It should be emphasized that improvement cannot be expected in all cases of P.N.H.Thus, glucocorticoids can be added to the group of drugs, including dextran given by intravenous infusion (Gardner and Laforet, 1960) and androgen plus iron therapy (Hartman et alit, 1966), which occasionally can be of benefit in the management of this disease.