Ring-like Corneal Deposits in a Case of Congenital Spherocytosis.

A girl aged 14 years was first seen at a local hospital on August 5, 1961. She had been ill for about 4 days with pyrexia, pallor, jaundice, and pain in the splenic region. She had had recurrent attacks of a similar nature since the age of 6 years. The spleen was enlarged reaching about two fingers below the costal margin, and there was a number of palpable shotty glands in the neck and groins. Haemoglobin 5 3 g./100 ml. (37 per cent.); colour index 1 0; white cell count 6,000/ml.; reticulocyte count 5 8 per cent. Many spherocytes seen in the blood film. Direct Coombs's test negative. Serum bilirubin 1 8 mg./100 ml., but other liver function tests normal. The sternal marrow showed active erythropoiesis consistent with a haemolytic anaemia. A urobilinogen test on the urine gave a moderately strong positive reaction. The blood of the patient's two sisters, parents, and maternal grandmother showed no anaemia, reticulocytosis, spherocytosis, or increased fragility. A diagnosis of congenital spherocytosis was considered, but the absence of family affection and the presence of enlarged glands were anomalous. Immediate treatment consisted of a transfusion of 2 pints of blood, a course of systemic tetracycline, and systemic steroids (starting with prednisolone 10 mg. three times a day) reducing to 5 mg. on the second day, and thereafter maintained at 5 mg. twice daily. The patient was referred to the department of haematology of the Manchester Royal Infirmary and was admitted to hospital on May 10, 1962, for investigation and treatment. At that time, she stated that her vision had been slightly blurred for about 3 weeks. This was particularly troublesome when reading, as the extremities of the letters appeared indistinct. Examination.-The visual acuity was 6/5 in each eye. There were bilateral ring-like corneal opacities (Figure, opposite). These were oval or flattened, with the long axes horizontal, and were situated slightly below and lateral to the centre of the cornea, right more than left. Slit-lamp microscopy showed that the opacities consisted of an aggregation of very fine reddish-brown granular deposits situated in the deeper layers of the corneal epithelium. The internal margin of each ring-like opacity was relatively clear-cut, whereas the density of the fine granular deposits decreased less steeply at the peripheral margin, and one had the impression that the deposits were proceeding from the limbus towards the central rings. Apart from the corneae, the optic media were clear, and the fundi were normal. At this time, the patient was neither pale nor jaundiced, and the spleen was not palpable. Haemoglobin 13 7 g./100 ml. (94 per cent.); red cells 4,580,000/ml., reticulocytes 22 4 per cent. Blood film showed many spherocytes and a polymorph leucocytosis. Fragility of red cells grossly excessive. Bone-marrow showed intense normoblastic activity. Serum iron 140 mg./100 ml. Direct