Hyperviscosity Syndrome Clinical Presentation
- Author: Thomas J Hemingway, MD, FACEP; Chief Editor: Barry E Brenner, MD, PhD, FACEP more...
The clinical presentation of hyperviscosity syndrome consists principally of 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 manifestation of hyperviscosity syndrome. Patients may present with any of the following:
Spontaneous gum bleeding
Persistent bleeding after minor procedures
Visual changes range from blurred vision to vision loss. Neurologic manifestations are frequent and varied, and may include the following:
Somnolence progressing to stupor and coma
Other manifestations may include heart failure, shortness of breath, hypoxia, fatigue, and anorexia. In fact, the clinician should have a high index of suspicion for hyperviscosity syndrome in patients with unexplained coma/altered mental status or unexplained shortness of breath, especially in those with an underlying hematologic disorder.
Blood hyperviscosity plays a role in the pathogenesis of several cardiovascular diseases, including hypertension, atherosclerosis,and the metabolic syndrome.
Physical findings are related to the major organ systems involved, as follows:
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 abnormalities, 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 jugular venous pressure, and hypoxia)
Less commonly, the hyperproliferative blood cell disorders such as the leukemias, myeloproliferative diseases, polycythemia, or thrombocytosis may be implicated for the increased viscosity caused by proliferation of their respective cellular components.
Adams BD, Baker R, Lopez JA, Spencer S. Myeloproliferative disorders and the hyperviscosity syndrome. Emerg Med Clin North Am. 2009 Aug. 27(3):459-76. [Medline].
Stone MJ, Bogen SA. Role of plasmapheresis in Waldenström's macroglobulinemia. Clin Lymphoma Myeloma Leuk. 2013 Apr. 13 (2):238-40. [Medline].
Kwaan HC. Hyperviscosity in plasma cell dyscrasias. Clin Hemorheol Microcirc. 2013. 55 (1):75-83. [Medline].
Chen LY, Wong PC, Noda S, Collins DR, Sreenivasan GM, Coupland RC. Polyclonal hyperviscosity syndrome in IgG4-related disease and associated conditions. Clin Case Rep. 2015 Apr. 3 (4):217-26. [Medline].
Behl D, Hendrickson AW, Moynihan TJ. Oncologic emergencies. Crit Care Clin. 2010 Jan. 26(1):181-205. [Medline].
Sloop G, Holsworth RE Jr, Weidman JJ, St Cyr JA. The role of chronic hyperviscosity in vascular disease. Ther Adv Cardiovasc Dis. 2015 Feb. 9 (1):19-25. [Medline].
Boudin L, Romeo E, Mavrovi E, Tsitsi Nding P, Blade JS, de Jaureguiberry JP, et al. [Bing-Neel syndrome: Report of 4 cases and literature review.]. Rev Med Interne. 2014 Jun 3. [Medline].
Khan UA, Shanholtz CB, McCurdy MT. Oncologic mechanical emergencies. Emerg Med Clin North Am. 2014 Aug. 32 (3):495-508. [Medline].
Ramos-Casals M, Stone JH, Cid MC, Bosch X. The cryoglobulinaemias. Lancet. 2012. 379(9813):348-360.
Thomas EL, Olk RJ, Markman M, et al. Irreversible visual loss in Waldenström's macroglobulinaemia. Br J Ophthalmol. 1983. 67:102-106.
Blade J, Rosinol L. Complications of multiple myeloma. Hematol Oncol Clin North Am. 2007 Dec. 21(6):1231-46, xi. [Medline].
Bloch KJ, Maki DG. Hyperviscosity syndromes associated with immunoglobulin abnormalities. Semin Hematol. 1973 Apr. 10(2):113-24. [Medline].
Blum W, Porcu P. Therapeutic apheresis in hyperleukocytosis and hyperviscosity syndrome. Semin Thromb Hemost. 2007 Jun. 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.
Drew MJ. Plasmapheresis in the dysproteinemias. Ther Apher. 2002 Feb. 6(1):45-52. [Medline].
Fahey JL, Barth WF, Solomon A. Serum hyperviscosity syndrome. JAMA. 1965 May 10. 192:464-7. [Medline].
Gertz MA, Kyle RA. Hyperviscosity syndrome. J Intensive Care Med. 1995 May-Jun. 10(3):128-41. [Medline].
Higdon ML, Higdon JA. Treatment of oncologic emergencies. Am Fam Physician. 2006 Dec 1. 74(11):1873-80. [Medline].
Hoffman R, et al. Therapy. 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. 1994 Jul-Aug. 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. 1975 Jan. 50(1):29-40. [Medline].
Mehta J, Singhal S. Hyperviscosity syndrome in plasma cell dyscrasias. Semin Thromb Hemost. 2003 Oct. 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. 2005 Oct. 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.
Stone MJ, Bogen SA. Evidence-based focused review of management of hyperviscosity syndrome. Blood. 2012 Mar 8. 119(10):2205-8. [Medline].
Zarkovic M, Kwaan HC. Correction of hyperviscosity by apheresis. Semin Thromb Hemost. 2003 Oct. 29(5):535-42. [Medline].