Hyperviscosity syndrome: Difference between revisions

(Format, headings, links for ddx, ref for primary dx, separation of tx by cause)
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==Background==
==Background==
*Increased serum viscosity due to increased blood products or immunoglobulins
*Increased serum viscosity due to:
*Causes: Waldenstrom's, IgA myeloma, polycythemia, leukemia
*#Increased blood products (polycythemia, thrombocytosis. leukemia)
*#Immunoglobulins (Waldenstrom's, IgA myeloma, multiple myeloma)
*Symptoms arise from poor capillary flow and organ congestion


==Symptoms==
==Clinical Features==
*headache, blurred vision, AMS, focal neuro deficits
*Symptoms of hyperviscosity can appear when one of the following occur:
*dyspnea, hypoxia, fever, fatigue
**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>
*mucosal bleeding
**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


==Diagnosis==
===Symptoms===
*Elev. serum viscosity (>5cP)
*Gen: fever, fatigue
*Severe leukocytosis
*CNS: headache, blurred vision, AMS, focal neuro deficits
*Globulin gap (Total protein - albumin) > 4
*Pulm: dyspnea, hypoxia
*Cardiac: chest pain, CHF
*Hem: mucosal bleeding
 
==Differential Diagnosis==
''Detailed DDx can be found on each page''
*[[Stroke]]
*[[Altered mental status]]
*[[Chest pain]]
*[[Hypoxemia]]
*[[Dyspnea]]


==Workup==
==Workup==
*Chem, CBC
*CBC
*Consider coags, UA
*Chem 10
*Coags
*Type and screen
*UA
*CT brain if neuro symptoms
*CT brain if neuro symptoms
*CXR
*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 need admission
*Hem/Onc consult
==See Also==


==Treatment==
==External Links==
*Plasmapharesis / Leukapharesis depending on cause
*If unavailable, phlebotomize 2-3 units, give 2-3 L IVF
*Supportive care


==Source==
==Sources==
*Tintinalli 7th Ed.
<references/>
*Hemingway T. Hyperviscosity Syndrome. Medscape 2015

Revision as of 14:32, 8 March 2015

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 need admission
  • 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.