The utility of the autohaemolysis test for children with congenital haemolytic anaemia.

Abstract

Autohaemolysis testing can assist the evaluation of haemolytic anaemia, but involves a cumbersome assay that is difficult to perform accurately. Because this test persists in clinical practice without clear indications or guidelines, we retrospectively reviewed our experience with autohaemolysis testing for children with suspected congenital haemolytic anaemia. Over 12 years, autohaemolysis without glucose was elevated for 38 of 39 children with congenital spherocytosis, while glucose corrected or reduced autohaemolysis in 33 of these patients. Autohaemolysis was elevated only once among seven other cases (four with congenital nonspherocytic haemolytic anaemia and three normal siblings). In three cases of congenital spherocytosis with equivocal osmotic fragility, autohaemolysis was abnormal and corrected with glucose in two. In our experience, autohaemolysis testing was helpful diagnostically in only two of 54 cases, so has only limited utility as a routine test for children with suspected congenital haemolytic anaemia.