Hyperviscosity syndrome in plasma cell dyscrasias

Area of Science:

• Hematology
• Oncology

Background:

• Hypergammaglobulinemia elevates serum viscosity, frequently leading to hyperviscosity syndrome.
• Monoclonal hypergammaglobulinemia, observed in multiple myeloma and Waldenström's macroglobulinemia, is a primary cause.
• Elevated viscosity stems from increased protein content, large molecular size, and abnormal immunoglobulin structure.

Purpose of the Study:

• To review the pathophysiology, clinical manifestations, and management of hyperviscosity syndrome.
• To highlight the differences in symptomatic hyperviscosity prevalence between Waldenström's macroglobulinemia and multiple myeloma.
• To outline therapeutic strategies for immediate symptom control and long-term disease management.

Main Methods:

• Literature review of hyperviscosity syndrome associated with plasma cell dyscrasias.
• Analysis of factors contributing to serum viscosity.
• Summary of clinical presentation and treatment modalities.

Main Results:

• Symptomatic hyperviscosity occurs more frequently in Waldenström's macroglobulinemia (10–30%) than multiple myeloma (2–6%).
• Symptoms manifest when serum viscosity significantly exceeds normal levels (e.g., IgM ≥ 3 g/dL).
• Common symptoms include constitutional, bleeding, ocular, neurological, and cardiovascular issues.

Conclusions:

• Hyperviscosity syndrome management requires prompt plasmapheresis for symptom control.
• Long-term treatment focuses on managing the underlying plasma cell disorder to reduce monoclonal protein production.
• Plasma exchange may serve as the primary symptomatic therapy for select elderly or debilitated patients.