From the Guidelines
Hyperviscosity syndrome in multiple myeloma requires immediate therapeutic plasma exchange (TPE) for patients with symptomatic hyperviscosity, followed by rapid initiation of anti-myeloma therapy. The treatment of hyperviscosity is aimed at preventing complications such as bleeding, loss of vision, and irreversible neurologic impairment 1.
Key Considerations
- Clinical manifestations of hyperviscosity include mucosal hemorrhage, visual abnormalities along with neurologic and cardiac features such as heart failure, seizures, vertigo, and diplopia 1.
- Serum viscosity levels do not correlate well with the patients’ symptoms or clinical signs, and treatment decisions should be based on clinical presentation rather than laboratory values alone 1.
- Automated plasma exchange requires replacement of about two-thirds of the patient's plasma volume with 5% human albumin solution or an equal mixture of albumin and 0.9% normal saline 1.
Treatment Approach
- Therapeutic apheresis procedures are relatively safe, with a 3% to 4% overall incidence of adverse effects that are mostly reversible 1.
- It is essential to repeat the procedure at scheduled intervals, generally on a daily basis for 3 to 5 days until the hyperviscosity has been corrected and chemotherapy is initiated 1.
- In the absence of other treatments, cessation of plasma exchange treatments will result in a recurrence of symptoms within 2-3 weeks 1.
Definitive Treatment
- Definitive treatment involves starting anti-myeloma therapy promptly, usually with regimens containing proteasome inhibitors, immunomodulatory drugs, and dexamethasone.
- Regular monitoring of paraprotein levels is essential to assess treatment response and detect recurrence of hyperviscosity.
From the Research
Hyperviscosity Proteinemia in Multiple Myeloma
- Hyperviscosity syndrome is a complication that can occur in multiple myeloma, particularly in IgM multiple myeloma, due to elevated levels of monoclonal proteins in the blood 2.
- The syndrome can cause symptoms such as visual impairment, and treatment options include plasma exchange, immunoadsorption, and double filtration plasmapheresis 2.
- The concentration and molecular configuration of the myeloma protein are important determinants of the presence or absence of the hyperviscosity syndrome 3.
- In patients with IgG myeloma, the hyperviscosity syndrome can occur due to high molecular weight polymers of the IgG proteins, but it can also occur in the absence of these polymers, depending on the concentration and molecular configuration of the myeloma protein 3.
Diagnosis and Management of Multiple Myeloma
- Multiple myeloma is a hematologic malignancy characterized by the presence of abnormal clonal plasma cells in the bone marrow, and it is diagnosed based on the presence of certain biomarkers and clinical features 4, 5.
- The diagnosis of multiple myeloma requires the presence of ≥10% clonal bone marrow plasma cells or a biopsy-proven plasmacytoma, plus evidence of one or more multiple myeloma defining events (MDE) 5.
- Treatment options for multiple myeloma include induction therapy with a combination of an injectable proteasome inhibitor, an oral immunomodulatory agent, and dexamethasone, followed by autologous hematopoietic stem cell transplantation and maintenance therapy with lenalidomide 4, 5.
- Lenalidomide is a second-generation immunomodulatory agent that has shown significant clinical benefits in the treatment of multiple myeloma, both as induction and maintenance therapy 6.
Treatment of Hyperviscosity Syndrome in Multiple Myeloma
- Immunoadsorption using columns containing recombinant single domain camelid antibody fragments as ligands can be effective in reducing monoclonal protein levels and alleviating symptoms of hyperviscosity syndrome in patients with IgM multiple myeloma 2.
- However, broad spectrum immunoadsorption columns may be only moderately effective compared to total plasma exchange or double filtration plasmapheresis, and may require repeated sessions to achieve adequate reduction in monoclonal protein levels 2.
- Measuring IgG levels may be a reasonable strategy for monitoring drug levels of monoclonal antibodies during immunoadsorption 2.