II. Causes

  1. Oncology related cases
    1. Acute Myelogenous Leukemia (AML), accounts for 20% of cases
    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

III. 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

IV. Signs

  1. Spontaneous bleeding from mucous membranes
  2. Fever in 80% of patients
  3. Pulmonary symptoms in 30% of cases
    1. Dyspnea
    2. Hypoxia
    3. Pulmonary Infiltrates (CXR) may be seen
  4. Vision change
    1. Hemorrhagic Retinal veins (appear as sausages, pathognomonic)
  5. Neurologic symptoms (e.g. Transient Ischemic Attack) in 40% of cases
    1. Headache
    2. Ataxia
    3. Vertigo
    4. Seizures
    5. Altered Level of Consciousness or confusion
    6. Peripheral Neuropathy

V. 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)
        1. Hyperleukocytosis with White Blood Cell Count >100,000 (>50,000 in some criteria)
      2. Red Blood Cells markedly increased (e.g. Polycythemia Rubra Vera)
    2. Other cell lines may be elevated
      1. Platelet Count falsely elevated
  2. Serum Electrolytes with Renal Function panel
    1. Hyperkalemia may be present
  3. Urinalysis
  4. Coagulation Panel (e.g. INR/PT, PTT)
  5. Serum viscosity: >4-5 centipoise (cP)
    1. Serum viscosity is normally near 1.4 cP
    2. Water viscosity is 1 cP

VI. Management

  1. Oncologic Emergency
    1. Immediate Consultation with hematology and oncology
  2. Temporize
    1. Aggressive Intravenous Fluids
    2. Respiratory support (including Endotracheal Intubation if needed)
    3. Avoid transfusion
    4. Empiric antibiotics
      1. Fever is present in 80% of patients who also have severe Leukocytosis on Chemotherapy
      2. Antibiotics are indicated to cover the differential diagnosis in this high risk group
  3. Decrease cellular load (cytoreduction)
    1. Multiple Myeloma
      1. Plasmapheresis
    2. Polycythemia
      1. Phlebotomy
    3. Leukostasis
      1. Induction Chemotherapy (first-line, only measure associated with reduced mortality)
      2. Leukapheresis
      3. Hydroxyurea
        1. May be indicated in asymptomatic patients
        2. Dosing 50-100 mg/kg/day

VII. Complications

VIII. Prognosis

  1. Untreated mortality approaches 40% in first week

IX. References

  1. Aurora and Herbert in Herbert (2013) EM:Rap 13(10): 1-4
  2. Long, Long and Koyfman (2020) Crit Dec Emerg Med 34(11): 17-24
  3. Higdon (2006) Am Fam Physician 74:1873-80 [PubMed]
  4. Higdon (2018) Am Fam Physician 97(11):741-8 [PubMed]
  5. Zuckerman (2012) Blood 120(10): 1993-2002 [PubMed]

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Related Studies

Ontology: Hyperviscosity syndrome (C0221030)

Definition (CSP) any syndrome associated with increased viscosity of the blood; in syndrome of serum hyperviscosity there is spontaneous bleeding and neurologic and ocular disorders; syndromes of polycythemic hyperviscosity is marked by retarded blood flow, organ congestion, reduced capillary perfusion, and increased cardiac effort; syndromes of sclerocythemic hyperviscosity comprise those in which the deformability of erythrocytes is impaired, as in sickle cell anemia.
Concepts Disease or Syndrome (T047)
SnomedCT 11888009
English HYPERVISCOSITY SYNDROME, hyperviscosity syndrome, hyperviscosity syndromes, Hyperviscosity syndrome, Reimann's syndrome, Hyperviscosity syndrome (disorder)
Italian Sindrome da iperviscosità
Japanese 過粘稠度症候群, カネンチョウドショウコウグン
Czech Hyperviskózní syndrom
Hungarian hyperviscositas syndroma
Spanish síndrome de Reimann, síndrome de hiperviscosidad (trastorno), síndrome de hiperviscosidad, Síndrome de hiperviscosidad
Portuguese Síndrome de hiperviscosidade
Dutch hyperviscositeitssyndroom
French Syndrome d'hyperviscosité sanguine
German Hyperviskositaetssyndrom

Ontology: Leukostasis (C0282548)

Definition (MSHCZE) Porucha mikrocirkulace vznikající v důsledku hromadění leukocytů, které jsou nadměrně zastoupeny v periferní krvi; zejm. při chronické myeloidní leukemii. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI) A disorder involving the aberrant infiltration and aggregation of leukocytes into the vasculature of the body. Leukostasis is typically detected in the brain and lungs of persons with leukemia. It requires substantial ablative modalities to both reduce the number of cells present and to ensure dispersion of the aggregates.
Definition (MSH) Abnormal intravascular leukocyte aggregation and clumping often seen in leukemia patients. The brain and lungs are the two most commonly affected organs. This acute syndrome requires aggressive cytoreductive modalities including chemotherapy and/or leukophoresis. It is differentiated from LEUKEMIC INFILTRATION which is a neoplastic process where leukemic cells invade organs.
Concepts Disease or Syndrome (T047)
MSH D018921
SnomedCT 30419000
English Leukostases, Leukostasis, Leukostasis Syndrome, Leukostasis Syndromes, Syndrome, Leukostasis, Syndromes, Leukostasis, Leukostasis [Disease/Finding], Leukostasis syndrome, Leukostasis (morphologic abnormality)
Swedish Leukostas
Czech leukostáza, Syndrom leukostázy, Leukostáza
Finnish Leukostaasi
Russian LEIKOSTAZ, LEIKOSTAZA SINDROM, ЛЕЙКОСТАЗ, ЛЕЙКОСТАЗА СИНДРОМ
Japanese 白血球停滞, ハッケッキュウテイタイショウコウグン, ハッケッキュウテイタイ, 白血球停滞症候群
Italian Sindrome leucostasica, Sindrome da leucostasi, Leucostasi
French Syndrome de leucostase, Leucoagglutination, Leucostase
Polish Zespół leukostazy, Leukostaza
Norwegian Leukostase
Dutch leukostase, leukostasesyndroom, Leukostase, Leukostasissyndroom
German Leukostasesyndrom, Leukostase-Syndrom, Leukostase
Hungarian Leukostasis, Leukostasis-szindróma
Spanish Leucostasia, Síndrome de leucostasia, leucoestasis (anomalía morfológica), leucoestasis, leucostasis (anomalía morfológica), leucostasis, Leucostasis, Síndrome Leucostásico
Portuguese Leucostase, Síndrome de leucostase, Leucostasia, Síndrome de Leucostasia