What is the most common cause of hyperviscosity syndrome?
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What is the most common cause of hyperviscosity syndrome?
Hypergammaglobulinemia is the most common cause of HVS, specifically the monoclonal condition Waldenstrom macroglobulinemia. More than 30% of all WM patients develop HVS at some point in their life because of the large star-shaped IgM pentamers that are highly viscous. Myelomas are the second leading cause of HVS.
Why does IgM cause hyperviscosity?
For any given level of paraprotein, IgM or IgA is more likely than IgG to cause hyperviscosity because the former circulate as pentamers or dimers, respectively, in contrast to monomeric IgG.
What level of IgM causes hyperviscosity?
IgM is pentameric and very large in size (970 kDA), and serum viscosity can increase significantly with IgM levels as low as 3 g/dL, and IgM levels of 6 g/dL or higher are associated with rapid development of hyperviscosity, with a median time to symptomatic HVS of 3 months (9, 10).
What is a complication of hyperviscosity syndrome?
Complications may occur if the condition is more severe or if your baby isn’t responding to treatment. These complications can include: stroke. kidney failure.
What are the symptoms of hyperviscosity syndrome?
Symptoms of Hyperviscosity
- Lethargy.
- Headaches.
- Deafness.
- Convulsions.
- Issues with sight.
- Loss of vision.
- Hypertension.
- Heart failure.
Is Waldenstrom Macroglobulinemia a non Hodgkin’s lymphoma?
Waldenstrom macroglobulinemia is considered a type of non-Hodgkin’s lymphoma. It’s sometimes called lymphoplasmacytic lymphoma.
What immunoglobulin is increased in Waldenstrom’s macroglobulinemia?
In Waldenström macroglobulinemia, these abnormal cells produce excess amounts of IgM , the largest of a type of protein known as an immunoglobulin; the overproduction of this large protein contributes to the condition’s name (macroglobulinemia).
What are the symptoms of hyperviscosity?
Hyperviscosity syndrome is a group of symptoms triggered by an increase in the viscosity of the blood. Symptoms of high blood viscosity include spontaneous bleeding from mucous membranes, visual disturbances due to retinopathy, and neurologic symptoms ranging from headache and vertigo to seizures and coma.
How do you treat hyperviscosity?
Plasmapheresis is the treatment of choice for initial management and stabilization of hyperviscosity syndrome (HVS) caused by the paraproteinemias (the majority of cases). Plasmapheresis is usually well tolerated and safe.
How is hyperviscosity syndrome diagnosed?
The diagnosis of hyperviscosity syndrome (HVS) is confirmed by measurement of elevated serum viscosity in a patient with characteristic clinical manifestations of HVS. No exact diagnostic cut-off exists for serum viscosity, as different patients will have symptoms at different values.
How do you test for hyperviscosity syndrome?
There is no single diagnostic test for HVS. As always, a good history and physical are important. Laboratory evaluation includes a complete blood count (CBC), peripheral blood smear, serum electrolytes and serum viscosity. CBC: patients with HVS are typically anemic due to their underlying disease.
Can Waldenstrom’s turn into multiple myeloma?
Certain changes in the DNA inside normal lymphocytes can cause them to become lymphoma or multiple myeloma cells. Changes in the DNA of some lymphoma cells can also cause them to produce high levels of IgM, leading to symptoms of Waldenstrom’s.
How high is IgM in Waldenstrom?
Smoldering Waldenström macroglobulinemia (also referred to as indolent or asymptomatic Waldenström macroglobulinemia) is defined as a serum IgM monoclonal protein level of 3 g/dL or higher and/or bone marrow lymphoplasmacytic infiltration of 10% or greater and no evidence of end-organ damage, such as anemia.
How do you treat Hyperviscosity?
Can Hyperviscosity be cured?
How quickly does Waldenstrom’s progress?
The cumulative probability of progression to symptomatic WM, amyloidosis, or lymphoma was 6% at 1 year, 39% at 3 years, 59% at 5 years, and 68% at 10 years. The major risk factors for progression were percentage of lymphoplasmacytic cells in the bone marrow, size of the serum M-spike, and the hemoglobin value.