From the heterogeneous group of the non-spherocytic haemolytic anaemias a new clinical and haematological entity has been isolated in recent years in which the presence of red-cell inclusion bodies and the excretion of dark brown urine are associated with the characteristics commonly observed in all chronic haemolytic processes.

In 1952 Cathie described what may be the first case in medical literature. He noted the remarkable finding of apparently spontaneous Heinz bodies in the erythrocytes of an infant after splenectomy. Later on a new characteristic was added to the pattern of the disease, the identification of dipyrrolic mesobilifuscin in the urine (Schmid, Brecher and Clemens, 1959), and more recently an abnormal haemoglobin fraction was detected (Scott, Haut, Cartwright and Wintrobe, 1960).

Up to now less than ten cases have been reported. The subject has been briefly and clearly reviewed by Schmid (1961).

We had the opportunity of studying an Italian family in which the mother and the only daughter were affected by the disease. It was fortunate for our study that only the mother had been splenectomized; we could, therefore, compare the haematological findings in a splenectomized and in a non-splenectomized case. The finding of an abnormal haemoglobin component makes our observation similar to those of Scott et al. (1960), Pribilla (1962) and Hutchison, Pinkerton, Waters, Douglas, Lehmann and Beale (1964).