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Management of Hyperviscosity Syndromes

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Abstract

The hyperviscosity syndromes are a group of hematologic disorders characterized by (among other symptoms) the classic triad of visual changes, mucosal bleeding, and neurologic deficits. They include disorders of WBCs (hyperleukocytosis), platelets (thrombocytosis), RBCs (erythrocytosis), and elevated protein levels (paraproteinemia). The symptoms of these disorders are the result of an increase in serum viscosity which leads to a relative hypoperfusion. This hypoperfusion can result in catastrophic end organ damage. In the setting of hyperviscosity syndromes secondary to excess WBCs, this is termed leukostasis. Leukostasis is diagnosed empirically when a patient presents with symptoms of tissue hypoxia and is found to have an underlying leukemia with a hyperleukocytosis (WBC >100,000 thou/mL). Leukostasis is a medical emergency and requires immediate stabilization of the patient, which can range from fluid resuscitation to mechanical ventilation. Signs of tumor lysis syndrome should be monitored for and close coordination with oncology is imperative. Ultimately, patients with hyperviscosity from leukocytosis will require a rapid reduction in WBC through a procedure called leukapheresis. Leukapheresis is a temporizing measure performed to decrease symptoms until the diagnosis is made and treatment of the underlying malignancy is initiated.

Keywords

  • Hyperviscosity syndrome
  • Hyperleukocytosis
  • Leukostasis
  • Paraproteinemia
  • Plasmapheresis
  • Leukapheresis
  • Tumor lysis syndrome
  • Waldenstroms macroglobulinemia
  • Acute myelogenous leukemia (AML)
  • Acute lymphoblastic leukemia (ALL)
  • Chronic myelogenous leukemia (CML)
  • Leukocyte larceny

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Correspondence to Brian P. O’Connor .

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O’Connor, B.P., Subramanian, I.M. (2017). Management of Hyperviscosity Syndromes. In: Hyzy, R. (eds) Evidence-Based Critical Care. Springer, Cham. https://doi.org/10.1007/978-3-319-43341-7_76

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  • DOI: https://doi.org/10.1007/978-3-319-43341-7_76

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