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Anaemia is a commonly encountered medical condition although retinopathy due to aplastic anaemia is not commonly seen.
A 23-year-old male presented with a 1-week history of bilateral blurred vision, worse in the left eye. It was associated with frontal and temporal headache made worse by coughing. There was no preceding trauma or blood loss, he was on no medication and was not exposed to noxious chemicals or irradiation. He was not diabetic nor hypertensive. Prior to developing blurred vision, he had coryzal-like symptoms for 2 days but no myalgia, rash or joint pains. His maternal uncle died of a blood disorder and his maternal cousin has acute lymphoid leukaemia.
On examination, he was non obese but looked pale. He was haemodynamically stable with a heart rate of 82 beats/min and a blood pressure of 150/56 mmHg. Visual acuity was 6/18 (right eye) and 6/60 (left eye). Anterior segments of both eyes were normal with no pupillary defect. Fundoscopy showed bilateral grossly swollen optic discs with scattered retinal haemorrhages more marked in the posterior pole and gross macular oedema in the left eye (Figure 1a,b).
A computerized tomography scan and magnetic resonance imaging of head and orbit showed no evidence of raised intracranial pressure, i.e. no space occupying lesion and normal ventricles. There were no meningeal infiltrations and optic nerves were normal. There were no features to suggest cerebral venous thrombosis.
Blood tests, including serum glucose, angiotensin converting enzyme, antineutrophilic cytoplasmic antibodies, syphilitic serology, anti-mitochondrial antibody, electrolytes and urea, folate and vitamin B12 were normal.
Full blood count revealed severe pancytopenia with haemoglobin of 5.8 g/dL, white cell count of 2.7×109/L, platelet count of 15×109/L and mean corpuscular volume of 109.8 fl. Blood film showed aplastic anaemia confirmed by a bone marrow biopsy.
He had packed cells and platelet transfusions initially and this was repeated due to insufficient endogenous haemopoiesis. He was subsequently commenced on antilymphocyte globulin and cyclosporin. As his haemoglobin improved, so did the retinopathy and visual acuity.
Ten months later, his haemoglobin stabilized at 15 g/dL with immunosuppressive treatment alone. The retinopathy had almost completely resolved with only a residual deep blotch haemorrhage at the left macula. His visual acuity was 6/5 right eye and 6/6 left eye.
Retinopathy associated with gastrointestinal haemorrhage and resolving following correction of the anaemia, was first reported by Ulrich in 1883.1 The manifestations of anaemic retinopathy are non-specific. Ophthalmic features include retinal haemorrhages, exudates, disc pallor, optic atrophy, neuritis, retinal detachment and papilloedema.1
The features of anaemic retinopathy seem to be universal, irrespective of the cause of the anaemia. The retinopathy also seems to resolve once the anaemia is corrected, as shown in the patient presented. It is widely believed that anaemia causes diminished capillary oxygenation, which increases the vessel wall permeability resulting in extravasation of blood products.2 There seems to be a direct correlation between the degree of anaemia and the severity of the retinopathy.
Aplastic anaemia is a life threatening condition. It usually presents with anaemia, bleeding and infection. It can be inherited but it is more commonly acquired. The ocular findings include cotton wool spots, nerve fibre layer or preretinal haemorrhages, vitreous haemorrhages and optic disc oedema.
Mansour et al. reported ocular findings in 18 patients with aplastic anaemia.3 In their report, the patients were known to be anaemic prior to the development of the retinopathy.
In our case, the patient certainly had disc oedema and nerve fibre layer haemorrhages with gross macular oedema, all of which resolved once the anaemia was corrected.
Some patients with aplastic anaemia also develop pseudotumour cerebi which may require treatment geared towards lowering the raised intracranial pressure.4-6 It has been suggested that papilloedema associated with aplastic anaemia could be due to increased intracranial pressure from anaemia-induced cerebral hypoxia.7
The presentation in this patient initially was suggestive of raised intracranial pressure. We feel it is important that clinicians are made aware of this unusual presentation and that this case might hopefully help direct treatment to the underlying cause.