Hyperviscosity syndrome: Difference between revisions

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==Clinical Features==
==Clinical Features==
*Symptoms of hyperviscosity can appear when one of the following occur:
**Red cell mass > 25% of normal<ref>Pearson TC and Messinezy M. The diagnostic criteria of polycythaemia rubra vera. Leuk Lymphoma. 1999; 22 (1):87-93.</ref>
**Plt >600,000/µL<ref>Murphy S et al. Diagnostic criteria and prognosis in polycythemia vera and essential thrombocythemia. Semin Hematol. 1999; 36(1):9-13.</ref>
**Leukocytosis >100,000
**Serum viscosity >4-5cP (normal 1.4-1.8cP)<ref>Mehta J and Singhal S. Hyperviscosity syndrome in plasma cell dyscrasias. Semin Thromb Hemost. 2003; 29(5):467-471.</ref> or Globulin gap (Total protein - albumin) > 4
===Symptoms===
*Gen: fever, fatigue
*Gen: fever, fatigue
*CNS: headache, blurred vision, AMS, focal neuro deficits
*CNS: headache, blurred vision, AMS, focal neuro deficits
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==Diagnosis==
==Diagnosis==
===Workup===
*CBC  
*CBC  
*Chem 10
*Chem 10
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**Due to "leukocyte larceny," "platelet larceny"<ref>Mehta A, Lichtin AE, Vigg A, Parambil JG. Platelet larceny: spurious hypoxaemia due to extreme thrombocytosis. Eur Respir J. 2008 Feb;31(2):469-72.</ref><ref>Sacchetti A, Grynn J, Pope A, Vasso S. Leukocyte larceny: spurious hypoxemia confirmed with pulse oximetry. J Emerg Med. 1990 Sep-Oct;8(5):567-9.</ref><ref>Lele AV, Mirski MA, Stevens RD. Spurious hypoxemia. Crit Care Med. 2005 Aug;33(8):1854-6.</ref>
**Due to "leukocyte larceny," "platelet larceny"<ref>Mehta A, Lichtin AE, Vigg A, Parambil JG. Platelet larceny: spurious hypoxaemia due to extreme thrombocytosis. Eur Respir J. 2008 Feb;31(2):469-72.</ref><ref>Sacchetti A, Grynn J, Pope A, Vasso S. Leukocyte larceny: spurious hypoxemia confirmed with pulse oximetry. J Emerg Med. 1990 Sep-Oct;8(5):567-9.</ref><ref>Lele AV, Mirski MA, Stevens RD. Spurious hypoxemia. Crit Care Med. 2005 Aug;33(8):1854-6.</ref>
**Excessive O2 consumption by WBCs or plts following blood sample collection
**Excessive O2 consumption by WBCs or plts following blood sample collection
===Evaluation===
*Symptoms of hyperviscosity can appear when one of the following occur:
**Red cell mass > 25% of normal<ref>Pearson TC and Messinezy M. The diagnostic criteria of polycythaemia rubra vera. Leuk Lymphoma. 1999; 22 (1):87-93.</ref>
**Plt >600,000/µL<ref>Murphy S et al. Diagnostic criteria and prognosis in polycythemia vera and essential thrombocythemia. Semin Hematol. 1999; 36(1):9-13.</ref>
**Leukocytosis >100,000
**Serum viscosity >4-5cP (normal 1.4-1.8cP)<ref>Mehta J and Singhal S. Hyperviscosity syndrome in plasma cell dyscrasias. Semin Thromb Hemost. 2003; 29(5):467-471.</ref> or Globulin gap (Total protein - albumin) > 4


==Management==
==Management==

Revision as of 00:15, 3 May 2016

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

  • 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

Diagnosis

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
  • Note: ABGs might have SaO2 vs. SpO2 discrepancies
    • Causing spurious hypoxemia on ABG, with low PaO2 (as opposed to dyshemoglobinemia)
    • Due to "leukocyte larceny," "platelet larceny"[1][2][3]
    • Excessive O2 consumption by WBCs or plts following blood sample collection

Evaluation

  • Symptoms of hyperviscosity can appear when one of the following occur:
    • Red cell mass > 25% of normal[4]
    • Plt >600,000/µL[5]
    • Leukocytosis >100,000
    • Serum viscosity >4-5cP (normal 1.4-1.8cP)[6] or Globulin gap (Total protein - albumin) > 4

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 if symptomatic
  • Hem/Onc consult

See Also

External Links

References

  1. ↑ Mehta A, Lichtin AE, Vigg A, Parambil JG. Platelet larceny: spurious hypoxaemia due to extreme thrombocytosis. Eur Respir J. 2008 Feb;31(2):469-72.
  2. ↑ Sacchetti A, Grynn J, Pope A, Vasso S. Leukocyte larceny: spurious hypoxemia confirmed with pulse oximetry. J Emerg Med. 1990 Sep-Oct;8(5):567-9.
  3. ↑ Lele AV, Mirski MA, Stevens RD. Spurious hypoxemia. Crit Care Med. 2005 Aug;33(8):1854-6.
  4. ↑ Pearson TC and Messinezy M. The diagnostic criteria of polycythaemia rubra vera. Leuk Lymphoma. 1999; 22 (1):87-93.
  5. ↑ Murphy S et al. Diagnostic criteria and prognosis in polycythemia vera and essential thrombocythemia. Semin Hematol. 1999; 36(1):9-13.
  6. ↑ Mehta J and Singhal S. Hyperviscosity syndrome in plasma cell dyscrasias. Semin Thromb Hemost. 2003; 29(5):467-471.
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