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Conclusions
Retinal manifestations of hyperviscosity syndrome occur at lower serum IgM and SV levels than previously reported. Indirect ophthalmoscopy with scleral depression along with retinal vessel diameter measurements are able to detect the earliest hyperviscosity syndrome–related complications and should be considered in the treatment of patients with WM.
Diagnosis
Retinal haemorrhages and retinal venous engorgement ('sausaging') can be seen on fundoscopy.
High IgM.
High plasma viscosity (if available; hyperviscosity syndrome rarely occurs below 4 cP).
Treatment
Plasma exchange.
Hyperviscosity syndrome
Caused by excessive levels of IgM.
Symptoms include:
Retinopathy leading to visual disturbance.
Neurological symptoms.
Skin and mucosal bleeding.
Cardiac failure (rare).
Each patient received a dilated fundus examination using slitlamp biomicroscopy and indirect ophthalmoscopy with scleral depression by an experienced retinal specialist. For the ophthalmoscopic examination, patients were in a recumbent position. Digital retinal photographs using a Topcon TRC-50EX system (Topcon Corporation, Tokyo, Japan) were taken to document the ophthalmoscopic findings. Hemorrhages were considered to be central when they appeared within a 40° field of view centered on the macula.
Hyperviscosity related retinopathy in waldenstrom's macroglobulinemia
Histological transformation
Transformation to diffuse large B-cell lymphoma occurs in 5-10% of patients.
It may be characterised by rapidly enlarging lymph nodes, extra-nodal disease and marked rise in serum lactate dehydrogenase.
Tissue biopsy is needed for diagnosis.