This protocol covers the clinical scenario of hyperviscosity syndrome presenting with the classic triad — mucosal hemorrhage, visual disturbance, and neurological signs — in patients where the initial course of therapeutic plasma exchange did not achieve the required viscosity and immunoglobulin reduction targets.

Clinical scenario

The presenting picture includes:

Mucosal hemorrhage — bilateral epistaxis, gingival, gastrointestinal, or retinal bleeding
Visual disturbance — retinal hemorrhage, papilledema, or blurred vision
Neurological manifestations — somnolence or coma, cerebral hemorrhage, seizure, or ataxia

Prior treatment — failure condition

The first-line step was therapeutic plasma exchange (therapeutic apheresis), initiated immediately. The targets were: reduction of plasma viscosity by 30–50% per session to safe levels, reduction of the serum immunoglobulin level by approximately 60% in a single exchange, resolution of mucosal hemorrhage, and improvement of retinopathy. When those goals are not reached, this next-line protocol applies.

Next-line approach

The next step involves systemic chemotherapy directed at reducing protein levels; the specific regimen and selection criteria are detailed in the full protocol.

References

The classic triad of hyperviscosity includes mucosal bleeding, visual abnormalities, and neurological abnormalities.
For patients with hemorrhagic or central nervous system manifestations, plasma exchange is the therapy of choice and is relatively safe.
The evidence suggests that bortezomib-based triplet regimens result in the fastest decline in protein levels and should be considered essential when hyperviscosity is seen in multiple myeloma.

DOI: 10.1182/blood-2018-06-846816

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Treatment of Hyperviscosity Syndrome with Bilateral Epistaxis and Mucosal Hemorrhage When Plasma Exchange Fails

Clinical scenario

Prior treatment — failure condition

Next-line approach

Treatment goal

References