HYPERLEUKOCYTOSIS

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HYPERLEUKOCYTOSIS
INTP - PPO, PHO, IAP. P5 15 slides
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DEFINITION HYPERLEUKOCYTOSIS

• Peripheral leukocyte count exceeding
  100,000/microliter
• Clinically significant Hyperleukocytosis
• WBC gt 200,000/ microliter in AML gt300,000 /
  microliter in ALL CML

INTP - PPO, PHO, IAP. P5 2/15
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INCIDENCE

• 9-13 ALL
• 5-22 AML
• 100 CML in chronic phase
• More common Infant ALL, AML, Tcell ALL with
  mediastinal mass, hypodiploid ALL.

INTP - PPO, PHO, IAP. P5 3/15
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CAUSE OF DEATH

• CNS Hemorrhage/ Thrombosis
• Pulmonary Leukostasis
• Metabolic Derangements

INTP - PPO, PHO, IAP. P5 4/15
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PATHOGENESIS

• Hyperleukocytosis -gt increases PCV
• Blood Viscosity increases
• Leukocyte cells aggregate
• Thrombi formation
• Myeloblasts-gt larger, stickier greater increase
  in viscosity

INTP - PPO, PHO, IAP. P5 5/15
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PATHOGENESIS

• High WBC Count -gt Leukemic aggregates proliferate
  in cerebral vasculature
• Damage to Vessel
• Hemorrhage

INTP - PPO, PHO, IAP. P5 6/15
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CLINICAL PRESENTAION OF CEREBRAL LEUKOCYTOSIS

• Asymptomatic
• Mental status changes
• Headaches
• Blurred vision
• Seizures, Coma
• Symptoms of stroke

INTP - PPO, PHO, IAP. P5 7/15
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CLINICAL PRESENTAION OF CEREBRAL LEUKCOCYTOSIS

• Papilledema
• Retinal artery or Retinal vein distension.
• A child with WBC gt100,000 evaluate for signs and
  symptoms of Hyperleukocytosis

INTP - PPO, PHO, IAP. P5 8/15
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CLINICAL PRESENATION OF PULMONARY LEUKOSTASIS

• Dyspnoea
• Hypoxia
• Acidosis
• Cyanosis
• Other features
• Priapism
• Clitoral engorgement
• Dactylitis

INTP - PPO, PHO, IAP. P5 9/15
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LABORATORY STUDIES

• Serum Electrolytes
• Uric Acid
• Renal Function tests
• Coagulation profile
• Chest radiograph

INTP - PPO, PHO, IAP. P5 10/15
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Therapy

• Intravenous hydration-3lts/m²/day. Adjust if
  raised ICP
• Alkalinization with sodium-bicarbonate
• Allopurinol

INTP - PPO, PHO, IAP. P5 11/15
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THERAPY

• Platelet count lt20,000/ microliter should receive
  platelet transfusion
• Hb level should not be above 10gm/dl
• Exchange transfusion or Leukopheresis

INTP - PPO, PHO, IAP. P5 12/15
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LEUKAPHERESIS

• Mean reduction 48 to 62 reduction.
• Need for anticoagulation
• Difficulty with access in small children.
• Limited availability in many hospitals.

INTP - PPO, PHO, IAP. P5 13/15
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THERAPY

• Exchange transfusion 52-66 mean reduction
• Systemic antileukemic therapy must be initiated
  early
• Prophylactic CNS irradiation 400cGy administered
  to prevent CNS hemorrhage-controversial

INTP - PPO, PHO, IAP. P5 14/15
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CONCLUSION

• Clinical Suspicion when WBC gt 100.000/ microliter
• Immediate therapeutic measures
• Early initiation of Anti Leukemic therapy

INTP - PPO, PHO, IAP. P5 15/15