Hyperviscosity Syndrome Treatment & Management
- Author: Thomas J Hemingway, MD, FACEP; Chief Editor: Barry E Brenner, MD, PhD, FACEP more...
No specific prehospital care is indicated for patients with hyperviscosity syndrome. Emergency medical services personnel should be attentive to airway, breathing, and circulation (the ABCs) and provide symptomatic support.
Emergency Department Care
Plasmapheresis is the treatment of choice for initial management and stabilization of hyperviscosity syndrome (HVS) caused by the paraproteinemias (the majority of cases). Plasmapheresis is usually well tolerated and safe. Leukapheresis, plateletpheresis, and phlebotomy are indicated for HVS from leukostasis, thrombocytosis, and polycythemia, respectively.
As plasmapheresis removes the circulating paraproteins, the serum viscosity decreases and symptoms improve. This procedure remains effective short-term treatment for HVS in the paraproteinemias because of the demonstrated correlation of paraprotein levels and serum viscosity and the 80% intravascular location of paraproteins, especially immunoglobulins (eg, in Waldenstr öm macroglobulinemia). As such, a relatively small reduction in the paraprotein concentration has a significant effect on lowering serum viscosity.
Because bleeding is the most common sign of HVS, urgent plasmapheresis using a cell separator should be carried out for patients experiencing visual symptoms, to reduce the likelihood of blindness from retinal hemorrhages/retinal detachment. Plasmapheresis can also reverse HVS-induced retinal changes promptly, including reducing retinal venous diameter and increased venous blood viscosity.
In similar fashion leukapheresis, plateletpheresis, and phlebotomy also decrease the serum viscosity by decreasing the existing cellular component in excess. Although these treatments are helpful in the acute phase, they typically do not alter the prognosis of the disease process, which is the underlying etiology. These diseases (eg, multiple myeloma, Waldenström macroglobulinemia, blood dyscrasias) should be definitively treated with the appropriate oncologic therapy, or the HVS will typically recur within a few weeks, requiring further pheresis.
Signs and symptoms of congestive heart failure (CHF) from hyperviscosity may not respond to standard therapies for CHF, and, in fact, can be exacerbated by dehydration from diuresis causing increased viscosity. However, plasmapheresis and/or cellular pheresis reverses these symptoms.
While arranging for plasmapheresis, treat hemorrhage, CHF, and metabolic imbalances with standard therapies. One caveat: Use caution with the decision to proceed with packed red blood cell transfusion (pRBCs) for minor bleeding because a single unit of pRBCs may increase the viscosity enough to cause worsening symptoms and clinical decompensation. If a transfusion is indicated, administer it by slow infusion.
If plasma/cellular pheresis is not readily available and the patient is decompensating, one may try vigorous intravenous hydration coupled with a 2-3 unit phlebotomy in the interim as a temporizing measure.
Upon commencing pheresis (especially leukapheresis) one should prepare for the possibility of tumor lysis syndrome and treat accordingly.
Ultimately, the underlying dysproteinemia or blood cell dyscrasia needs to be addressed, as plasmapheresis and similar therapies do not alter the underlying disease process. The definitive treatment varies according to the diagnosis but often involves chemotherapeutic agents, such as alkylating agents or nucleoside analogs, which should be addressed with the consulting hematologist/oncologist to prevent further deterioration and possible recurrent episodes.
A hematologist should be consulted to arrange plasma/cellular pheresis and plan for interval chemotherapy as indicated.
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].