Hyperviscosity syndrome

Background

  • Increased serum viscosity due to:
    1. Increased blood products (polycythemia, thrombocytosis. leukemia)
    2. Immunoglobulins (Waldenstrom's, IgA myeloma, multiple myeloma)
  • Symptoms arise from poor capillary flow and organ congestion

Clinical Features

  • Symptoms of hyperviscosity can appear when one of the following occur:
    • Red cell mass > 25% of normal[1]
    • Plt >600,000/µL[2]
    • Leukocytosis >100,000
    • Serum viscosity >4-5cP (normal 1.4-1.8cP)[3] or Globulin gap (Total protein - albumin) > 4

Symptoms

  • Gen: fever, fatigue
  • CNS: headache, blurred vision, AMS, focal neuro deficits
  • Pulm: dyspnea, hypoxia
  • Cardiac: chest pain, CHF
  • Hem: mucosal bleeding

Differential Diagnosis

Detailed DDx can be found on each page

Workup

  • CBC
  • Chem 10
  • Coags
  • Type and screen
  • UA
  • CT brain if neuro symptoms
  • CXR
  • Consider initiating inpatient testing with: Total protein, albumin, SPEP, UPEP, IgA, IgG, IgM

Management

  • ABCs
  • IVF and supportive care
  • Ig mediated
    • Plasmapharesis
  • Leukostasis
    • Leukopheresis
  • Thrombocytosis
    • Plateletpheresis
    • Consider ASA
  • Polycythemia
    • Phlebotomy
  • If pheresis is not unavailable, phlebotomize 2-3 units, give 2-3 L IVF

Disposition

  • Typically require admission is symptomatic
  • Hem/Onc consult

See Also

External Links

Sources

  1. ↑ Pearson TC and Messinezy M. The diagnostic criteria of polycythaemia rubra vera. Leuk Lymphoma. 1999; 22 (1):87-93.
  2. ↑ Murphy S et al. Diagnostic criteria and prognosis in polycythemia vera and essential thrombocythemia. Semin Hematol. 1999; 36(1):9-13.
  3. ↑ Mehta J and Singhal S. Hyperviscosity syndrome in plasma cell dyscrasias. Semin Thromb Hemost. 2003; 29(5):467-471.
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