From the Guidelines
Hyperproteinemia in hyperviscosity syndrome of multiple myeloma patients is typically diagnosed when serum total protein levels exceed 8.5 g/dL, with IgM paraprotein levels above 3 g/dL or IgG/IgA levels above 4 g/dL, although the severity of symptoms correlates better with serum viscosity than with absolute protein levels 1. The diagnosis of hyperproteinemia in hyperviscosity syndrome is crucial, as it can lead to severe complications such as bleeding, visual disturbances, and neurological symptoms.
Key Diagnostic Criteria
- Serum total protein levels exceeding 8.5 g/dL
- IgM paraprotein levels above 3 g/dL or IgG/IgA levels above 4 g/dL
- Serum viscosity measurements above 4.0 centipoise (normal range 1.4-1.8 cp)
- Clinical presentation, including the classic triad of bleeding, visual disturbances, and neurological symptoms
Laboratory Assessment
- Serum protein electrophoresis
- Immunofixation
- Quantitative immunoglobulins to identify and measure the monoclonal protein
- Peripheral blood smear to detect rouleaux formation
- Elevated ESR The management of hyperviscosity syndrome requires prompt recognition and treatment, as it represents a medical emergency.
Treatment
- Immediate plasma exchange to reduce paraprotein levels
- Appropriate antimyeloma therapy to address the underlying disease
- Repeat plasma exchange procedures at scheduled intervals, generally on a daily basis for 3 to 5 days, until the hyperviscosity has been corrected and chemotherapy is initiated 1.
From the Research
Hyperproteinemia Criteria in Hyperviscosity Syndrome of Multiple Myeloma Patients
- The criteria for hyperproteinemia in hyperviscosity syndrome of multiple myeloma patients are not explicitly defined in the provided studies.
- However, according to the study 2, symptomatic hyperviscosity usually appears when the normal serum viscosity of 1.4 to 1.8 cp reaches 4 to 5 cp, corresponding to a serum immunoglobulin M (IgM) level of at least 3 g/dL, an IgG level of 4 g/dL, and an IgA level of 6 g/dL.
- The study 3 reports a case of hyperviscosity syndrome due to IgA multiple myeloma, where the patient received therapeutic plasma exchange (TPE) treatments, resulting in symptomatic improvement and a decrease in IgA and viscosity levels.
- The study 4 discusses the efficacy of plasmapheresis in improving blood flow properties in patients with hyperviscosity syndrome, including a patient with multiple myeloma, and reports a significant reduction in paraprotein concentrations and plasma viscosity after plasmapheresis.
Diagnosis and Management of Multiple Myeloma
- The study 5 provides an overview of the diagnosis and management of multiple myeloma, including the evaluation of patients with possible multiple myeloma, which includes measurement of serum protein electrophoresis with immunofixation.
- The study 6 discusses the role of plasmapheresis in the treatment of paraproteinemia, including multiple myeloma, and reports that plasmapheresis is used in combination with corticosteroids and immunosuppressive drugs to prevent production of abnormal proteins or to treat the underlying disease.
Treatment of Hyperviscosity Syndrome
- The studies 4, 2, and 3 suggest that plasmapheresis or therapeutic plasma exchange (TPE) is an effective treatment for hyperviscosity syndrome in multiple myeloma patients, as it can reduce paraprotein concentrations and plasma viscosity, leading to symptomatic improvement.
- The study 2 reports that immediate therapy of symptomatic hyperviscosity is directed at reduction of blood viscosity by plasmapheresis to control symptoms, while long-term management is directed at control of the underlying disease to prevent production of the monoclonal protein.