Department of Internal Medicine, Gelre Ziekenhuizen, location Lukas Hospital, 7300 DS APELDOORN, The Netherlands.
A 53-year-old woman without significant medical history was admitted to our outpatient clinic with recurrent haemorrhages in the peri-orbital region of both eyes (Fig. 1). This patient was sent by the ophthalmologist who had found no intra-orbital lesions. The haemorrhages occurred spontaneously approximately three times a month without preceding trauma. On physical examination no abnormalities (in particular no haemorrhages) were observed. Laboratory investigation revealed normal platelet counts and no clotting disorders were found.
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A diagnostic procedure was performed.
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A biopsy of the kidney showed granular mesangial depositions which stained positive for Congo red, suggesting these depositions to be amyloid fibrils. These depositions could also be seen in the HE-section (Fig. 2). There were no observations suggestive of a glomerulonephritis. There only was a small amount of focal glomerulosclerosis.
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Peri-orbital ecchymoses in AL amyloidosis is called raccoon eyes or raccoon syndrome [1,2], and is caused by vascular infiltration of amyloid fibrils in peri-orbital blood vessels. These ecchymoses may occur spontaneously (as in our patient) or after minimal trauma such as sneezing or rubbing the eyes. Raccoon eyes are rather specific for AL amyloidosis. Other causes of peri-orbital bleeding include clotting disorders, essential thrombocytaemia and trauma. There is no consequence for the vision as there is no intra-ocular problem.
In the treatment of AL amyloidosis, chemotherapy consisting of melphalan and prednisolone may be considered [3,4] in patients with an adequate pre-treatment performance status. Combination therapy of intravenous melphalan and autologous haematopoetic cell transplantation may be of benefit in patients with involvement of fewer than two major organ systems, uncomplicated cardiac symptomatology and patients younger than 50 years [5]. Successful treatment with the iodinated anthracycline 4'-iodo-4'-deoxydoxorubicin has also been described [6]. There is no specific treatment for the raccoon eyes. Reduction of the proteinuria might be achieved using ACE-inhibitors [7] or NSAIDS [8].
Unfortunately, during the last 2 years our patient was admitted several times because of dyspneu and oedema of all extremities. Chest radiographs showed pleural effusions. Treatment with diurestics, an angiotensin-II-receptor antagonist and cyclosporin has not been very successful, she needs high doses of loop-diuretics to prevent increase of pleural effusions. Moreover, there is a subjective intolerance to most drugs resulting in poor compliance.
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