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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

Waldenström's macroglobulinaemia (WM) is a chronic, indolent, B-cell lymphoproliferative disorder characterised by an IgM monoclonal paraprotein and bone marrow infiltration by lymphoplasmacytic cells (lymphoplasmacytic lymphoma – LPL

The clinical manifestations are numerous and are caused by tumour infiltration of bone marrow and organs as well as the effects of the IgM paraprotein which can lead to hyperviscosity syndrome, amyloidosis and peripheral neuropathy.

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.

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.

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