HVS due to paraproteinemia in patients with WM is a life-threatening emergency requiring urgent treatment with plasmapheresis. In HVS, elevated viscosity is due to entrapment of high levels of IgM in the intravascular space. To lower serum viscosity, half or more of the intravascular volume must be removed. Plasmapheresis is a temporary solution that must be repeated every 1-2 weeks, and viscosity levels should be assessed before and after each plasmapheresis. The plasma removed during plasmapheresis is replaced with albumin and saline.
Chemotherapy agents inhibit the release of paraproteins, decreasing symptoms and risks for hyperviscosity and related issues. Therefore, chemotherapy is recommended as soon as possible after plasmapheresis to decrease the risk of morbidity and mortality.
Signs and symptoms of HVS include spontaneous bleeding, dizziness, headache, blurry vision due to retinopathy, and hearing problems. Gastrointestinal symptoms include malabsorption, bleeding, and diarrhea.
Neither treatment with multiple IV boluses of normal saline nor enoxaparin therapy is an appropriate treatment option for HVS. As previously stated, HVS is a medical emergency, not a minor complication of WM.
Learn more about HVS.
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Cite this: Emmanuel C. Besa. Fast Five Quiz: Can You Treat Waldenström Macroglobulinemia? - Medscape - Mar 22, 2022.