Hyperviscosity Syndrome 

  • Author: Thomas J Hemingway, MD; Chief Editor: Barry E Brenner, MD, PhD, FACEP   more...
 
Updated: Jul 8, 2010
 

Background

Hyperviscosity syndrome (HVS) refers to the clinical sequelae of increased blood viscosity. Increased serum viscosity usually results from increased circulating serum immunoglobulins and can be seen in such diseases as Waldenström macroglobulinemia and multiple myeloma. It can also result from increased cellular blood components (typically white or red blood cells) in hyperproliferative states such as the leukemias, polycythemia, and the myeloproliferative disorders.

The complications most commonly associated with this syndrome include mucous membrane bleeding, neurologic and pulmonary symptoms, and the associated retinopathy.

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Pathophysiology

Viscosity is a property of liquid and is described as the resistance that a liquid exhibits to the flow of one layer over another. As serum proteins or cellular components increase, the blood becomes more viscous, leading to the clinical symptoms of hyperviscosity syndrome secondary to the vascular stasis and resultant hypoperfusion.

The normal relative serum viscosity ranges from 1.4-1.8 units (reported as Centipoises). Symptoms usually are not seen at viscosities of less than 4 units, and the hyperviscosity syndrome typically requires a viscosity greater than 5 units.

Hyperviscosity syndrome (HVS) is associated most commonly with plasma cell dyscrasias (the paraproteinemias) and is due to the large size of the excess immunoglobulin M (IgM) paraproteins in these disorders. Waldenström macroglobulinemia is the most common cause and accounts for about 85% of cases of HVS. Less frequently, the disease can occur in multiple myeloma (especially with myeloma proteins of the IgA and IgG3 types) and connective tissue diseases.

Hyperviscosity syndrome can also be caused by the bone marrow hyperproliferative states: the leukemias, polycythemia, essential thrombocytosis, and the myelodysplastic disorders, which also increase serum viscosity.

Confusion and mental status changes result from the increased viscosity of the blood and decreased cerebral blood flow. This sludging leads to segmental dilatation of retinal veins and retinal hemorrhages. Mucosal bleeding may occur from prolonged bleeding time caused by myeloma proteins interfering with platelet function.

Cardiopulmonary symptoms such as shortness of breath, hypoxemia, acute respiratory failure, and hypotension also result from this sludging of blood and decreased microvascular circulation.

Clinical sequelae of HVS can include congestive heart failure, ischemic acute tubular necrosis, and pulmonary edema with multiorgan system failure and death if treatment is not promptly initiated.[1] Thus, prompt recognition and expeditious treatment are imperative in preventing deterioration.[2]

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Epidemiology

Mortality/Morbidity

Mortality is related to the underlying cause of the hyperviscosity syndrome.

Sex

While not much information is available regarding the incidence of hyperviscosity syndrome, one study found that 61% of blood dyscrasias occur in males.

Age

Little information is available regarding the age of patients with hyperviscosity syndrome. Most blood dyscrasias are not diagnosed until the seventh decade of life.

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Contributor Information and Disclosures
Author

Thomas J Hemingway, MD  Attending Physician, Department of Emergency Medicine, Wilcox Memorial Hospital

Thomas J Hemingway, MD is a member of the following medical societies: American College of Emergency Physicians

Disclosure: Nothing to disclose.

Coauthor(s)

Eric Alexander Savitsky, MD  Associate Clinical Professor of Medicine, Department of Medicine, Division of Emergency Medicine, University of California at Los Angeles Medical Center

Eric Alexander Savitsky, MD is a member of the following medical societies: Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Douglas F Kupas, MD  Associate Chief Academic Officer, Geisinger Health System; Assistant Dean for Medical Student Affairs, Temple University Geisinger Clinical Campus; Commonwealth EMS Medical Director, Pennsylvania Department of Health

Douglas F Kupas, MD is a member of the following medical societies: American College of Emergency Physicians, National Association of EMS Physicians, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Specialty Editor Board

Robin R Hemphill, MD, MPH  Associate Professor, Director, Quality and Safety, Department of Emergency Medicine, Emory University

Robin R Hemphill, MD, MPH is a member of the following medical societies: American College of Emergency Physicians and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Jeffrey L Arnold, MD, FACEP  Chairman, Department of Emergency Medicine, Santa Clara Valley Medical Center

Jeffrey L Arnold, MD, FACEP is a member of the following medical societies: American Academy of Emergency Medicine and American College of Physicians

Disclosure: Nothing to disclose.

John D Halamka, MD, MS  Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center

John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Chief Editor

Barry E Brenner, MD, PhD, FACEP  Professor of Emergency Medicine, Professor of Internal Medicine, Program Director, Emergency Medicine, University Hospitals, Case Medical Center

Barry E Brenner, MD, PhD, FACEP is a member of the following medical societies: Alpha Omega Alpha, American Academy of Emergency Medicine, American College of Chest Physicians, American College of Emergency Physicians, American College of Physicians, American Heart Association, American Thoracic Society, Arkansas Medical Society, New York Academy of Medicine, New York Academy of Sciences, and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

References

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  10. Hussein M. Multiple myeloma: an overview of diagnosis and management. Cleve Clin J Med. Jul-Aug 1994;61(4):285-98. [Medline].

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  12. Kyle RA. Multiple myeloma: review of 869 cases. Mayo Clin Proc. Jan 1975;50(1):29-40. [Medline].

  13. Mehta J, Singhal S. Hyperviscosity syndrome in plasma cell dyscrasias. Semin Thromb Hemost. Oct 2003;29(5):467-71. [Medline].

  14. Ovadia S, Lysyy L, Floru S. Emergency plasmapheresis for unstable angina in a patient with hyperviscosity syndrome. Am J Emerg Med. Oct 2005;23(6):811-2. [Medline].

  15. Rogers R. Emergencies in Hematology and Oncology - Subtle and Atypical presentations, pearls and pitfalls. EMedHome.com [web site].

  16. Rosen P, et al. Marx: Rosen's Emergency Medicine: Concepts and Clinical Practice. 5th ed. Mosby Inc; 2002.

  17. Zarkovic M, Kwaan HC. Correction of hyperviscosity by apheresis. Semin Thromb Hemost. Oct 2003;29(5):535-42. [Medline].

 
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