Hyperviscosity Syndrome When Systemic Chemotherapy Has Not Achieved the Required IgM Reduction
This protocol applies to patients with hyperviscosity who have an indication for therapy and no symptoms, but in whom prior rapid-acting systemic chemotherapy has not met the serum IgM reduction targets that would otherwise eliminate the need for further intervention.
Clinical Scenario
Hyperviscosity with an indication for therapy and no symptoms. In this setting, rapid-acting systemic chemotherapy is the first approach — it can reduce serum viscosity and remove the need for further treatment, but only when specific IgM reduction goals are reached.
Why Escalation to This Protocol Is Triggered
The previous line used rapid-acting systemic chemotherapy — bortezomib-based therapy, carfilzomib-based therapy, or ibrutinib — targeting a meaningful reduction in serum IgM level. An IgM reduction of as little as 25% is sufficient to eliminate symptoms; defined deeper reduction targets within set timeframes were not achieved. Failure to reach these serum IgM reduction goals is the specific trigger for escalation to this protocol.
Next-Step Approach (Partial Overview)
When measured viscosity reaches a defined threshold, a plasma exchange–based intervention (therapeutic apheresis) represents the evidence-based next step, with session-level plasma viscosity reduction as the defined treatment goal. Full eligibility criteria, procedural parameters, and session-level targets are in the complete protocol.
References
DOI: 10.1182/blood-2018-06-846816
- For patients who have hyperviscosity, an indication for therapy, and no symptoms, rapid-acting systemic chemotherapy can reduce the serum viscosity and obviate the need for plasma exchange.
- The standard of care for managing hyperviscosity of the plasma is therapeutic apheresis, and guidelines on the use of apheresis have been previously published, and hyperviscosity is considered category A evidence for plasma exchange.
- Plasma exchange will decrease the plasma viscosity anywhere from 30% to 50% in a single session that exchanges 1 volume of the patient's plasma.