Hyperviscosity Syndrome Treatment & Management

Updated: Feb 21, 2018
  • Author: Thomas J Hemingway, MD, FACEP; Chief Editor: Barry E Brenner, MD, PhD, FACEP  more...
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Treatment

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. [12]

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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. [13]

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 Waldenström macroglobulinemia). As such, a relatively small reduction in the paraprotein concentration has a significant effect on lowering serum viscosity. [10]

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. [14]

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. [6]

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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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