UC Irvine

A 30 YEAR old South African woman of British extraction was investigated because of a persistent reticulocytosis. As a child she had been chronically anaemic, and at the age of six her spleen had been removed. After that time she had been well, but on one occasion she became “ill” after a course of sulphonamide. She was found to have a well compensated haemolytic anaemia; the haemoglobin level was 13 g/100 ml. of blood, and 5–8 per cent of her red cells were reticulocytes. Though of normal size the red cells showed target cell formation, basophilic stippling, and 75–80 per cent contained Heinz bodies. The test for abnormally heat labile haemoglobin1 was positive. The patient's mother was found to carry the same haemoglobin abnormality but was not thought to suffer from severe haemolysis. She did, however, experience an episode of severe haemoglobinuria following sulphonamides.