Coags

Hyperviscosity Syndrome

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Hyperviscosity Syndrome, Leukostasis

  • Causes
  1. Oncology related cases
    1. Acute Myelogenous Leukemia (AML)
    2. Chronic Myelogenous Leukemia (CML) if in blast crisis, evolution to AML
    3. Multiple Myeloma
    4. Waldenstrom's Macroglobulinemia (10-30% of cases)
  2. Other causes
    1. Polycythemia Vera
    2. Sickle Cell Anemia
    3. Sepsis
  • Pathophysiology
  1. Overall increased serum viscosity
  2. Dramatically increased cell counts
    1. Red Blood Cells: Polycythemia Rubra Vera
    2. White Blood Cells: Leukostasis (with White Blood Cells >100,000)
      1. Especially seen in acute leukemic blast crisis (AML or CML)
  3. Increased circulating serum Immunoglobulins
    1. Immunoglobulins coat Red Blood Cells
    2. Results in increased viscosity and sludging of blood cells
    3. End result is end organ decreased perfusion
  • Signs
  1. Spontaneous bleeding from mucous membranes
  2. Pulmonary symptoms
    1. Dyspnea
    2. Pulmonary Infiltrates (CXR) may be seen
  3. Vision change
    1. Hemorrhagic Retinal veins (appear as sausages, pathognomonic)
  4. Neurologic symptoms (e.g. Transient Ischemic Attack)
    1. Headache
    2. Ataxia
    3. Vertigo
    4. Seizures
    5. Altered Level of Consciousness or confusion
    6. Peripheral Neuropathy
  • Labs
  1. Complete Blood Count
    1. Marked increased in a cell line
      1. White Blood Cells markedly increased or Hyperleukocytosis (e.g. Acute Myelogenous Leukemia)
      2. Red Blood Cells markedly increased (e.g. Polycythemia Rubra Vera)
  2. Serum viscosity: >4-5 centipoise (cP)
    1. Serum viscosity is normally near 1.4 cP
    2. Water viscosity is 1 cP
  • Management
  1. Oncologic Emergency
    1. Immediate Consultation with hematology and oncology
  2. Temporize
    1. Intravenous Fluids
    2. Respiratory support (including intubation if needed)
    3. Avoid transfusion
  3. Decrease cellular load
    1. Multiple Myeloma: Plasmapheresis
    2. Leukostasis: Leukapheresis
    3. Polycythemia: Phlebotomy