eMedicine Specialties > Emergency Medicine > Hematology & Oncology
Hyperviscosity Syndrome
Updated: Sep 12, 2008
Introduction
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.
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 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.
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.
Clinical
History
Clinical symptoms generally are related to the triad of mucosal bleeding, visual changes, and neurologic symptoms. Constitutional symptoms and cardiorespiratory symptoms also contribute to the clinical presentation.
- The tendency to bleed is the most common symptom of hyperviscosity syndrome.
- Spontaneous gum bleeding
- Epistaxis
- Rectal bleeding
- Menorrhagia
- Persistent bleeding after minor procedures
- Visual changes range from blurred vision to vision loss.
- Neurologic manifestations are frequent and varied. The neurologic symptoms of hyperviscosity have been referred to as the Bing-Neal syndrome.
- Vertigo
- Hearing loss
- Paresthesias
- Ataxia
- Headaches
- Seizures
- Somnolence progressing to stupor and coma
- Other manifestations may include heart failure, shortness of breath, hypoxia, fatigue, and anorexia.
- In fact, one should have a high index of suspicion for HVS in patients with unexplained coma/altered mental status or unexplained shortness of breath especially in those with an underlying hematologic disorder.
Physical
Physical findings are related to the major organ systems involved.
- Bruises, epistaxis, or gum bleeding may be noted.
- Ophthalmic examination may reveal decreased visual acuity, dilated retinal veins, "sausage-linked" or "boxcar segmentation" of the retinal veins, or retinal hemorrhages.
- Neurologic examination may reveal various findings, including diminished mental status, confusion, ataxia, or nystagmus.
- Cardiopulmonary examination may reveal signs of congestive heart failure with volume overload (rales, lower extremity edema, elevated JVP, and hypoxia).
Causes
Increased serum viscosity usually results from increased circulating serum immunoglobulins and can be seen in Waldenström macroglobulinemia and multiple myeloma.
Less commonly, the hyperproliferative blood cell disorders such as the leukemias, myeloproliferative diseases, polycythemia, and thrombocytosis may be implicated for the increased viscosity caused by proliferation of their respective cellular components.
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References
Bladé J, Rosiñol L. Complications of multiple myeloma. Hematol Oncol Clin North Am. Dec 2007;21(6):1231-46, xi. [Medline].
Bloch KJ, Maki DG. Hyperviscosity syndromes associated with immunoglobulin abnormalities. Semin Hematol. Apr 1973;10(2):113-24. [Medline].
Blum W, Porcu P. Therapeutic apheresis in hyperleukocytosis and hyperviscosity syndrome. Semin Thromb Hemost. Jun 2007;33(4):350-4. [Medline].
D'Alessio T, Kupas DF. Altered mental status in a 57-year-old woman with multiple myeloma. Top Emerg Med. 1996;18(2):72-8.
Fahey JL, Barth WF, Solomon A. Serum hyperviscosity syndrome. JAMA. May 10 1965;192:464-7. [Medline].
Higdon ML, Higdon JA. Treatment of oncologic emergencies. Am Fam Physician. Dec 1 2006;74(11):1873-80. [Medline].
Hoffman R, et al. Therapy. In: Meloni D, Cox KJ, eds. Hematology: Basic Principles and Practice. 4th ed. Philadelphia, Pa: Elsevier, Churchill, Livingstone; 2005.
Hussein M. Multiple myeloma: an overview of diagnosis and management. Cleve Clin J Med. Jul-Aug 1994;61(4):285-98. [Medline].
Kwaan HC, Bongu A. The hyperviscosity syndromes. Semin Thromb Hemost. 1999;25(2):199-208. [Medline].
Kyle RA. Multiple myeloma: review of 869 cases. Mayo Clin Proc. Jan 1975;50(1):29-40. [Medline].
Mehta J, Singhal S. Hyperviscosity syndrome in plasma cell dyscrasias. Semin Thromb Hemost. Oct 2003;29(5):467-71. [Medline].
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].
Rogers R. Emergencies in Hematology and Oncology - Subtle and Atypical presentations, pearls and pitfalls. EMedHome.com [web site].
Rosen P, et al. Marx: Rosen's Emergency Medicine: Concepts and Clinical Practice. 5th ed. Mosby Inc; 2002.
Zarkovic M, Kwaan HC. Correction of hyperviscosity by apheresis. Semin Thromb Hemost. Oct 2003;29(5):535-42. [Medline].
Further Reading
Keywords
hyperviscosity syndrome, HVS, increased blood viscosity, increased serum viscosity, mucous membrane bleeding, retinopathy, Waldenström macroglobulinemia, multiple myeloma, leukemias, polycythemia, myeloproliferative disorders, plasma cell dyscrasias, paraproteinemias
