From the Guidelines
Hyperviscosity syndrome is diagnosed based on a combination of clinical symptoms and laboratory findings, including serum viscosity greater than 4.0 centipoise, visual disturbances, neurological symptoms, and bleeding, with treatment initiated for symptomatic patients, particularly those with hyperviscosity, neuropathy, or other indicative symptoms 1. The criteria for hyperviscosity syndrome include:
- Serum viscosity greater than 4.0 centipoise (normal is 1.4-1.8)
- Clinical manifestations such as:
- Visual disturbances (blurred vision, retinal hemorrhages)
- Neurological symptoms (headaches, dizziness, confusion)
- Bleeding (epistaxis, gingival bleeding)
- Laboratory findings showing elevated protein levels, particularly in conditions like:
- Waldenström macroglobulinemia (IgM > 3 g/dL)
- Multiple myeloma (IgA or IgG > 4 g/dL)
- Polycythemia (hematocrit > 60%) According to the NCCN guidelines, treatment should be initiated for patients with a diagnosis of WM/LPL only in those who are symptomatic, with indicative symptoms including hyperviscosity, neuropathy, symptomatic adenopathy or organomegaly, amyloidosis, cryoglobulinemia, cold agglutinin disease, anemia, B symptoms, and presence of cytopenia 1. Management typically involves plasmapheresis for immediate relief of symptoms, followed by treating the underlying condition causing the hyperviscosity, such as chemotherapy for plasma cell dyscrasias or phlebotomy for polycythemia 1. Key considerations in the management of hyperviscosity syndrome include:
- Initiating treatment for symptomatic patients, particularly those with hyperviscosity or other indicative symptoms
- Using plasmapheresis for immediate relief of symptoms
- Treating the underlying condition causing the hyperviscosity
- Avoiding agents that limit future treatment options, such as continuous oral alkylator therapy or nucleoside analogues, particularly in younger patients who may be potential SCT candidates 1.
From the Research
Hyperviscosity Syndrome Criteria
The criteria for hyperviscosity syndrome include:
- Clinical manifestations such as neurological impairment, visual disturbance, and bleeding 2
- Measurement of plasma or serum viscosity by a viscometer to assess diagnosis 2
- Funduscopic examination to identify abnormalities correlated with abnormal plasma viscosity 2
- Etiologies including various conditions, with symptomatic hyperviscosity more common in Waldenström's macroglobulinemia and multiple myeloma 2
Diagnosis and Treatment
Diagnosis and treatment of hyperviscosity syndrome involve:
- Viscosity measurements to confirm diagnosis, although therapy can be initiated without these measurements if clinical presentation is typical 3
- Plasma exchange as the therapy of choice for patients with hemorrhagic or central nervous system manifestations 3
- Phlebotomy for patients with extreme elevation of red cell and white cell count 3
- Therapeutic apheresis, including plasmapheresis and cytapheresis, to remove abnormal plasma components or excess blood cells 4
Associated Conditions
Hyperviscosity syndrome is associated with various conditions, including: