Hyperviscosity Syndrome
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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. [1] HVS 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 clinical presentation in HVS consists principally of the triad of mucosal bleeding, visual changes, and neurologic symptoms. [2] Constitutional symptoms and cardiorespiratory symptoms may also occur.
The diagnosis of HVS is confirmed by measurement of elevated serum viscosity in a patient with characteristic clinical manifestations of HVS. No exact diagnostic cut-off exists for serum viscosity, however, as different patients will have symptoms at different values.
Plasmapheresis is the treatment of choice for initial management and stabilization of HVS from elevated immunoglobulin levels. [3] Leukapheresis, plateletpheresis, and phlebotomy are indicated for HVS from leukostasis, thrombocytosis, and polycythemia, respectively.
Definitive treatment of HVS is treatment of the underlying disorder (eg, chemotherapy). If the underlying disease process is left untreated, the hyperviscosity will recur.
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. Vascular stasis and resultant hypoperfusion then lead to the clinical symptoms of hyperviscosity syndrome (HVS).
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 HVS typically requires a viscosity greater than 5 units.
HVS is associated most commonly with plasma cell dyscrasias [4] (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, HVS can occur in multiple myeloma (especially with myeloma proteins of the IgA and IgG3 types) and connective tissue diseases. Chen and colleagues describe seven cases of polyclonal HVS caused by IgG4-related disease. [5]
HVS can also be caused by the bone marrow hyperproliferative states, as follows:
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. [6] Thus, prompt recognition and expeditious treatment are imperative in preventing deterioration. [2]
Little epidemiologic information is available on hyperviscosity symdrome. One study found that 61% of blood dyscrasias occur in males. Most blood dyscrasias are not diagnosed until the seventh decade of life. Mortality is related to the underlying cause of the hyperviscosity syndrome.
Stone MJ, Bogen SA. Evidence-based focused review of management of hyperviscosity syndrome. Blood. 2012 Mar 8. 119(10):2205-8. [Medline].
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].
Rajagopal R, Apte RS. Seeing through thick and through thin: Retinal manifestations of thrombophilic and hyperviscosity syndromes. Surv Ophthalmol. 2016 Mar-Apr. 61 (2):236-47. [Medline].
Hernández-Molina G, Bermúdez-Bermejo P. Hyperviscosity in primary Sjögren’s syndrome: clinical implications. Int J Rheum Dis. 2017 Jan. 20 (1):84-89. [Medline].
Caimi G, Carlisi M, Urso C, Lo Presti R, Hopps E. Clinical disorders responsible for plasma hyperviscosity and skin complications. Eur J Intern Med. 2017 Jul. 42:24-28. [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. Hematol Oncol Clin North Am. 2017 Dec. 31 (6):927-940. [Medline].
Zarkovic M, Kwaan HC. Correction of hyperviscosity by apheresis. Semin Thromb Hemost. 2003 Oct. 29(5):535-42. [Medline].
Thomas EL, Olk RJ, Markman M, et al. Irreversible visual loss in Waldenström’s macroglobulinaemia. Br J Ophthalmol. 1983. 67:102-106.
Thomas J Hemingway, MD, FACEP Attending Physician, Department of Emergency Medicine, Wilcox Memorial Hospital
Thomas J Hemingway, MD, FACEP is a member of the following medical societies: American College of Emergency Physicians
Disclosure: Nothing to disclose.
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, Society for Academic Emergency Medicine
Disclosure: Securisyn Medical – serve on Advisory Board and own shares (<5%).
Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Received salary from Medscape for employment. for: Medscape.
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, American College of Physicians
Disclosure: Nothing to disclose.
Barry E Brenner, MD, PhD, FACEP Professor of Emergency Medicine, Professor of Internal Medicine, Program Director for Emergency Medicine, Sanz Laniado Medical Center, Netanya, Israel
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, New York Academy of Medicine, New York Academy of Sciences, Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.
Robin R Hemphill, MD, MPH Associate Professor, Director, Quality and Safety, Department of Emergency Medicine, Emory University School of Medicine
Robin R Hemphill, MD, MPH is a member of the following medical societies: American College of Emergency Physicians, Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.
Hyperviscosity Syndrome
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