Sir,

Acute myeloid leukaemia may manifest itself in the eyes in many ways. Retinal involvement is the most common. Cutaneous lesions have also been described but periorbital cutaneous infiltration is rare. We report an unusual case which illustrates simultaneous involvement of periorbital skin and retinal vasculature by acute myeloid leukaemia. Palliative radiotherapy to the infiltrated eyelids produced effective symptomatic relief.

Case report

A 77-year-old Chinese woman was referred after a routine optician check showed flame haemorrhages along the superonasal arcade in the left eye suggesting superonasal branch retinal vein occlusion. She was otherwise asymptomatic with vision of 6/18 in both eyes. Anterior segments and intraocular pressures were normal. There were bilateral moderate cataracts.

Baseline investigations including full blood count, urea and electrolytes, blood glucose, lipid profile, clotting, erythrocyte sedimentation rate and immunoglobulin screen were performed. This revealed a raised white cell count of 17.3 × 109/1 with monocytosis. Urgent bone marrow biopsy showed reduced erythropoiesis and marked increase in primitive monocytoid cells with foamy blue-grey cytoplasm and cleaved nuclei. This confirmed the diagnosis of chronic myelomonocytic leukaemia in transformation into acute myeloid leukaemia.

Further ophthalmic assessment was requested when the patient developed periorbital swelling 2 weeks later. Examination showed bilateral upper and lower lid diffuse swelling causing narrowing of the palpebral fissures (Figure 1). The overlying skin was indurated and tender to touch. There was bilateral conjunctival chemosis. Vision in the left eye was reduced to 6/60. The remainder of the examination was unremarkable.

Figure 1
figure 1

Anterior segment photograph of the right eye showing diffuse swelling of the upper and lower lids causing narrowing of the palpebral fissure.

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An urgent CT scan showed bilateral severe soft tissue thickening around the anterior globes but intraorbital structures and optic nerves were normal (Figure 2). Biopsy of the periorbital skin showed diffuse infiltration of the dermis by cells with folded nuclei and pale cytoplasm (Figure 3a). Occasional cells showed positivity with chloroacetate esterase in keeping with a myeloid leukaemic infiltrate (Figure 3b). The patient received palliative chemotherapy with etoposide. Palliative radiotherapy to the eyelids with 10 Gray divided into two treatments resulted in reduction of eyelid swelling, enabling better eye opening. Vision in the left eye returned to 6/18.

Figure 2
figure 2

Axial cut of CT scan of the orbits showing bilateral severe soft tissue thickening around the anterior globes and normal intraorbital structures.

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Figure 3
figure 3

(a) Periorbital skin biopsy (low magnification) showing diffuse infiltration of the dermis by cells with folded nuclei and pale cytoplasm. (b) Periorbital skin biopsy (high magnification) showing occasional cells staining positive with chloroacetate esterase, in keeping with a myeloid leukaemic infiltrate.

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Comment

Retinal vein occlusion is a significant cause of visual impairment in the middle-aged and elderly population. Various risk factors have been identified, including hypertension,1,2 hyperlipidaemia,3 diabetes mellitus3 and hyperviscosity syndromes.4 This case illustrates the importance of investigating for such risk factors in patients with retinal vein occlusion, where hyperviscosity secondary to leukaemia is found.

Ophthalmic involvement is frequently seen in leukaemia.5,6,7 The retina is most commonly affected, either as a result of direct infiltration or secondary to changes in blood viscosity and clotting status.7 Cutaneous lesions have also been well described.6,8,9 Involvement of the periorbital skin however is rare.6,10,11 Treatment of cutaneous lesions with radiation is effective in producing symptomatic relief.11 This modality of treatment is of particular value in providing symptomatic relief in terminally ill patients while avoiding eyelid surgery.

This case illustrates the different ways in which acute myeloid leukaemia can manifest itself in the eye. It also demonstrates the efficiency of radiotherapy as a palliative treatment for subcutaneous eyelid leukaemic infiltration.