Incredibly grateful to the @CPSolvers team for letting me present another #VMR case (#86). Hopefully they won't get sick of @RezidentMD and me 😂. Below are a few teaching points related to the final diagnosis. #spoileralert
@RosenelliEM @rabihmgeha @Sharminzi @ArsalanMedEd
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@RosenelliEM @rabihmgeha @Sharminzi @ArsalanMedEd
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Symptomatic hyperviscosity is a medical emergency that requires prompt plasmapheresis. So although the diagnosis is established by measuring serum viscosity, this takes time to come back so initiate plasmapheresis on the basis of the patient's symptoms and physical findings
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What are those findings? Many, most non-specific, but neuro heavy: Oronasal bleeding, blurry vision, headaches, dizziness, paresthesias, retinal vein engorgement and flame-shaped hemorrhages, papilledema, nystagmus, deafness, diplopia, ataxia, dyspnea, stupor, and coma.
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Although the correlation between serum viscosity and clinical manifestations is not precise, symptoms often begin when serum viscosity is >4 cP, and most are symptomatic when serum viscosity is >6 cP. But if your patient is symptomatic, treat regardless of the number.
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Higher serum IgM levels at presentation are associated with a shorter time to the development of symptomatic hyperviscosity. Specifically, serum IgM level >6000 mg/dL was associated with a median time to symptomatic hyperviscosity of three months in one study.
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Though most of these patients are anemic, unless acutely indicated try to avoid RBC transfusion prior to plasmapheresis as this can increase serum viscosity and potentially worsen symptoms. It can also precipitate heart failure 😳
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Why does hyperviscosity occur in WM? As @AnnKumfer mentioned in the chat, IgM is a pentamer. Large amounts of IgM aggregate and via their carbohydrate chains, bind to H2O and increase osmotic pressure and resistance to blood flow.
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In addition, immunoglobulins can interact with RBCs and reduce their ability to travel through microvasculature. This can lead to decreased perfusion and tissue damage, manifesting as the aforementioned symptoms.
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How does plasmapheresis work? By removing IgM! A typical prescription is a total plasma volume exchange, replaced with albumin, repeated daily until symptoms subside or until serum viscosity is normal.
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However, plasmapheresis does treat the disease process. In most, IgM levels will rise and symptoms of HV will recur within a few weeks of stopping plasmapheresis unless therapy for WM is initiated. Most regimens include Rituximab. Get your Heme-Onc friends on board!
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Hopefully you enjoyed the case. Would love some additional teaching from @Anand_88_Patel, @fernandbteich, or others. Thanks again! Excellent discussion by all!
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