First Author: A.F.Miranda PORTUGAL
Co Author(s): J. Cardoso N. Marques A. Cardoso S. Barros P. Telles N. Campos 0 0 0 0 0 0 0 0 0
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Waldenstr̈̈om macroglobulinemia (WM) is a rare but severe lymphoproliferative B-cell disorder characterized by overproduction of monoclonal IgM and is the most frequent diagnosis in patients with hyperviscosity syndrome (HVS) caused by paraproteinemias. It is more frequent in elderly men. Vascular disturbances play a major role in HVS and can especially be observed in the retina with dilated veins, hemorrhage at all retinal levels and cotton wool spots. We describe a patient with severe HVS as a result of WM and unilateral vitreous hemorrhage due to vascular disturbance.
Hospital Garcia de Orta, Portugal.
A 45-year-old man without any relevant medical history, presented to the emergency room with difused cerebral edema and intracranial hypertension due to HVS. Systemic evaluation showed increased lymphadenopathy and hepatosplenomegaly, microcytic anemia (haemoglobin of 7,4 g/dL), normal platelet count, prolonged prothrombin and partial thromboplastin time, periferal blood with 49% lymphoid cells and rouleaux. Bone marrow aspiration and biopsy diagnosed WM as the cause of the HVS. Four days after his admission, the patient referred myodesopsias and decresed visual acuity (VA) in his right eye (RE).
Ophthalmologic observation revealed VA in RE of 0,2 and VA in LE of 1,0; intraocular pressure (IOP) was 12 mmHg in both eyes; anterior segment examination was normal; fundus examination revealed a vitreous hemorrhage in the RE preventing retina examination and in the LE dilated and tortuous veins, multiple retinal hemorrhages, severe atrophy of retinal pigment epithelium and optic disc edema. B-scan ocular ultrasound showed no signs of retinal tear or detachment in the RE. The patient was treated with plasmapheresis with significant improvement of his condition.
This is a case of unusual severe ocular and systemic first manifestations of HVS. The retinal findings and the vitreous hemorrhage were caused by vascular disturbances that occur in patients with this syndrome.