Treatment of Hyperviscosity Syndrome in Symptomatic Cryoglobulinemia

When hyperviscosity syndrome arises in the setting of symptomatic cryoglobulinemia, prompt intervention is needed to reduce circulating cryoglobulin levels and relieve symptoms before peripheral vascular complications progress.

Clinical Scenario

This protocol applies to patients with symptomatic type 1 or type 2 cryoglobulinemia presenting with hyperviscosity. Types 1 and 2 cryoglobulins drive hyperviscosity through a temperature-dependent gelling phenomenon in the peripheral circulation — rapidly raising serum viscosity. Type 2 cryoglobulins are also rheumatoid factors present in high titer.

Treatment Approach

The structured protocol involves an extracorporeal removal procedure used as primary therapy to directly reduce cryoglobulin burden — with specific procedural requirements to account for temperature sensitivity. The complete step-by-step regimen, including procedural specifications, is available in the full protocol.

Treatment goal: Reduction of circulating cryoglobulin (cryoprotein) levels with improvement in symptoms.

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References

DOI: 10.1182/blood-2018-06-846816

Types 1 and 2 cryoglobulinemia (type 2 cryoglobulins are also rheumatoid factors present in high titer) are well-described causes of hyperviscosity as the gelling phenomenon that occurs in the peripheral circulation rapidly raises the viscosity of serum and is highly temperature dependent.

Plasmapheresis can be used as primary therapy for reduction of cryoglobulin levels in cases of symptomatic essential cryoglobulinemia.

The procedure was carried out at room temperature with reinfusion through a blood warmer.

Improvement in symptoms was associated with removal of the cryoprotein.

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