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Hyperviscosity Syndrome Treatment & Management

  • Author: Thomas J Hemingway, MD, FACEP; Chief Editor: Barry E Brenner, MD, PhD, FACEP  more...
 
Updated: Nov 23, 2015
 

Prehospital Care

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.[8]

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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.[9]

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.[10]

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.[5]

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Consultations

A hematologist should be consulted to arrange plasma/cellular pheresis and plan for interval chemotherapy as indicated.

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Contributor Information and Disclosures
Author

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.

Coauthor(s)

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%).

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.

Specialty Editor Board

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.

Chief Editor

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

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

Disclosure: Nothing to disclose.

Additional Contributors

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.

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