From the Guidelines
Hyperviscosity syndrome in multiple myeloma requires prompt treatment with plasmapheresis followed by anti-myeloma therapy. The treatment of hyperviscosity is aimed at preventing complications such as bleeding, loss of vision, and irreversible neurologic impairment 1. Plasmapheresis should be initiated immediately when patients present with symptoms such as visual disturbances, neurological abnormalities, or bleeding due to high paraprotein levels. This procedure rapidly reduces serum viscosity by removing excess monoclonal proteins from circulation. Typically, 1-2 plasma volumes are exchanged per session, with daily treatments until symptoms resolve, as recommended by the American Society of Apheresis 1.
Following plasmapheresis, definitive treatment of the underlying multiple myeloma is essential, usually with a combination regimen such as VRd (bortezomib, lenalidomide, and dexamethasone) or alternative regimens like KRd (carfilzomib, lenalidomide, dexamethasone) or DRd (daratumumab, lenalidomide, dexamethasone). The choice of regimen may depend on patient-specific factors and the presence of other complications. Hydration is crucial during treatment to maintain adequate blood flow and prevent further complications. Regular monitoring of serum viscosity, paraprotein levels, and symptom assessment guides the need for additional plasmapheresis sessions.
The use of plasmapheresis in conjunction with systemic therapy is also supported by guidelines for the treatment of Waldenström's macroglobulinaemia, which can also present with hyperviscosity syndrome, emphasizing the importance of prompt intervention to manage symptoms and prevent long-term damage 1. The initiation of plasmapheresis should not be delayed, as it can rapidly improve symptoms and prevent complications, making it a critical component of the management of hyperviscosity syndrome in multiple myeloma. Key considerations in the management include:
- Prompt initiation of plasmapheresis for symptomatic patients
- Use of a combination anti-myeloma regimen following plasmapheresis
- Regular monitoring of serum viscosity and paraprotein levels
- Maintenance of adequate hydration to prevent further complications
- Consideration of patient-specific factors in the choice of anti-myeloma regimen.
From the Research
Treatment of Hyperviscosity Syndrome in Multiple Myeloma
- The primary treatment for hyperviscosity syndrome in multiple myeloma is plasmapheresis, which reduces blood viscosity and provides symptomatic relief 2, 3, 4, 5.
- Plasmapheresis has been shown to be effective in reducing paraprotein concentrations, plasma viscosity, and whole blood viscosity, resulting in improved blood flow properties and microcirculation 4.
- Therapeutic plasma exchange (TPE) is also used to treat hyperviscosity syndrome, and its efficiency in removing IgA, IgG, and IgM has been reported 4, 5.
- In addition to plasmapheresis and TPE, treatment of the underlying disease, such as multiple myeloma, is crucial to prevent the production of monoclonal proteins and reduce the risk of hyperviscosity syndrome 2, 6.
- The prognosis of hyperviscosity syndrome in newly diagnosed multiple myeloma is generally poor, with a median overall survival of 3.6 years, and is associated with high lethality in older patients 6.
Symptoms and Diagnosis
- Hyperviscosity syndrome typically presents with symptoms such as constitutional symptoms, bleeding, and ocular, neurological, and cardiovascular manifestations when serum viscosity reaches 4 to 5 cp 2.
- The diagnosis of hyperviscosity syndrome is based on the measurement of serum viscosity, paraprotein concentrations, and clinical symptoms 2, 3, 4, 5.
Management and Outcome
- The management of hyperviscosity syndrome in multiple myeloma involves a combination of plasmapheresis, TPE, and treatment of the underlying disease 2, 3, 4, 5, 6.
- The outcome of patients with hyperviscosity syndrome in multiple myeloma is generally poor, with a high risk of early death, especially in older patients 6.