• Leukostasis/hyperleukocytosis
  • Hyperleukocytosis: WBC count > 100,000/μL (typically in AML)
  • Leukostasis:  intravascular accumulation of WBCs/blasts in vascular lumen (+/- fibrin) resulting in end-organ damage
  
  

  Posted 11/16/16 10:53:02 AM by Matthew Cummings

• Leukostasis: Clinical Overview
  • End-organ damage results from vascular occlusion
  • Eyes and lungs most common sites for vascular obstruction
    • Extremities, kidneys, heart, can also be affected. 
  • Diagnosis? AML patient p/w  dyspnea or AMS w/ WBC is > 100,000/μL in the absence of a clear etiology
    • Common ddx: PNA/neutropenic sepsis, TRALI/TACO (if recent products)
  • Emergent management: Cytoreduction w/ leukopheresis +/- hydroxyurea (may also consider steroids for TRALI, broad-spectrum abx)
    • Indication for leukopheresis: clinical leukostasis OR asymptomatic w/ markedly elevated WBC to prevent sx
      • Leukopheresis: Typically two sessions (12-24 hours apart, each session 4-5 hours long)
      • Complications: hypotension (volume shifts), allergy to citrate (anti-coagulation in circuit), catheter-placement related
  
  

  Posted 11/16/16 11:02:20 AM by Matthew Cummings

• Pancytopenia w/ suspected leukemia? More likely to be AML > ALL
  
  

  Posted 11/16/16 11:03:52 AM by Matthew Cummings

• Tumor Lysis Syndrome Diagnosis
  • Primary: onset prior to initiation of rx due to high turnover of malignant cells
  • Secondary: shortly after the beginning of treatment (more common)
  • Diagnosis: 2+ abnormal values of uric acid (high), potassium (high), phosphorus (high), or calcium (low) at presentation OR 25% change in values from the pre-treatment values. 
    • Increased risk w/ high tumor burden, potential for rapid cell lysis (steroids), and underlying renal dysfunction
  
  

  Posted 11/16/16 11:19:10 AM by Matthew Cummings

• Tumor Lysis Management = Aggressive hydration, electrolyte correction, and reduction of uric acid levels 
  • IVF to maintain UOP typically 80 to 100 ml per hour (can add loop diuretics if low UOP, urinary alkalinization not needed)
  • HyperK/hyperPO4 = typical mgmt (latter add sevelamer)
  • AVOID administering calcium UNLESS symptomatic hypocalcemia (risk increasing Ca-PO4 product = CaPO4 renal crystallization) 
  • Reduce uric acid 
    • Allopurinol: xanthine oxidase inhibitor = prevents the formation of uric acid (takes 1-3d to lower) --> does not decrease the level of uric acid already present 
    • Rasburicase ("Recombinant urate oxidase") = reduces the level of uric acid by promoting the catabolism of uric acid to allantoin 
      • Typically used in established TLS (not as prevention) w/ presence of AKI, UA>9, or if not responding to allopurinol. 
      • G6PD deficiency is contraindication to rasburicase: H2O2 (rasburicase byproduct), cannot be broken down --> hemolysis/methemoglobinemia 
  
  

  Posted 11/16/16 11:19:44 AM by Matthew Cummings

• For further reading...

  How I treat hematologic emergencies in adults with acute leukemia (Blood)
  How I treat hyperleukocytosis in acute myeloid leukemia (Blood)
  Tumor Lysis Syndrome (NEJM Current Concepts)
  
  
  
  

  Posted 11/16/16 11:21:29 AM by Matthew Cummings

Created by Christopher Kelly

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