What are the criteria for hyperproteinemia in hyperviscosity syndrome?

From the Guidelines

Hyperproteinemia in hyperviscosity syndrome is typically diagnosed when serum IgM levels exceed 3 g/dL, along with serum viscosity greater than 4.0 centipoise, as indicated by the most recent guidelines 1. The diagnosis of hyperproteinemia in hyperviscosity syndrome depends on the underlying cause, with specific criteria varying for different conditions.

• For IgM-related hyperviscosity, such as in Waldenström macroglobulinemia, serum viscosity greater than 4.0 centipoise (normal range 1.4-1.8) along with IgM levels above 3 g/dL often indicates clinically significant hyperviscosity.
• The NCCN guidelines 1 recommend treatment initiation for patients with symptomatic hyperviscosity, and immediate plasmapheresis is recommended for patients requiring immediate disease control, such as those with symptomatic hyperviscosity.
• Clinical suspicion should increase when patients present with the classic triad of bleeding (especially mucosal), visual disturbances, and neurological symptoms alongside elevated protein levels.
• Laboratory evaluation should include serum protein electrophoresis, immunofixation, and direct measurement of serum viscosity when available, as noted in the guidelines 1.
• The relationship between protein levels and hyperviscosity is not strictly linear, as protein structure and polymerization significantly affect blood viscosity beyond simple concentration.
• Treatment should be considered in asymptomatic patients with serum IgM level >6,000 mg/dL, as recommended by the NCCN Panel 1.
• The management of hyperproteinemia in hyperviscosity syndrome typically involves plasma exchange to rapidly reduce protein levels when symptomatic hyperviscosity is present, followed by treatment of the underlying condition causing the hyperproteinemia, with consideration of the latest guidelines 1.

From the Research

Hyperproteinemia Criteria in Hyperviscosity Syndrome

The criteria for hyperproteinemia in hyperviscosity syndrome are not explicitly defined in the provided studies. However, the following points can be inferred:

• Hyperproteinemia is a key factor in the development of hyperviscosity syndrome, as it increases blood viscosity 2.
• The syndrome is characterized by a triad of symptoms: mucosal hemorrhages, visual disturbances, and neurological disorders 2.
• Laboratory tests can identify the etiology of the syndrome, with Waldenström's macroglobulinemia being the most common cause, followed by multiple myeloma and cryoglobulinemias 2.
• Serum viscosity correctly predicts the presence of clinical hyperviscosity syndrome in patients with stable hemoglobin and albumin concentrations 3.
• Funduscopic examination is a key exam because abnormalities are well-correlated with abnormal plasma viscosity 4, 2.

Key Findings

• Hyperviscosity syndrome is a life-threatening complication that requires prompt treatment 4, 2.
• Therapeutic plasma exchange is the primary treatment for severe cases or following confirmation by fundoscopy 4, 2.
• Targeted treatment of the underlying cause is implemented following plasma exchange sessions to prevent recurrence 2.
• The management of hyperviscosity syndrome should be primarily directed at the removal of the abnormal component, accompanied by measures that can control the production of the causative element 5.

Relevant Studies

• 6 discusses the role of plasmapheresis in the treatment of paraproteinemia, including hyperviscosity syndrome.
• 3 studies the clinical and laboratory indices of hyperviscosity in patients with paraproteinemia.
• 4 and 2 provide an overview of hyperviscosity syndrome, its clinical manifestations, diagnosis, and treatment.
• 5 discusses the pathophysiology and management of hyperviscosity syndromes.