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Key Features

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  • Approximately 500 new cases occur per year in children and adolescents in the United States

  • Although AML accounts for only 25% of all leukemias in this age group, it is responsible for at least one-third of deaths from leukemia in children and teenagers

  • Congenital conditions associated with an increased risk of AML include

    • Diamond-Blackfan anemia

    • Neurofibromatosis

    • Down syndrome

    • Wiskott-Aldrich, Kostmann

    • Li-Fraumeni syndromes

    • Chromosomal instability syndromes such as Fanconi anemia

  • Acquired risk factors include exposure to ionizing radiation, cytotoxic chemotherapeutic agents, and benzenes

  • However, the vast majority of patients have no identifiable risk factors

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Clinical Findings

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  • Clinical manifestations commonly include anemia (44%), thrombocytopenia (33%), and neutropenia (69%)

  • Symptoms may be few and innocuous or may be life threatening

  • Hyperleukocytosis may be associated with life-threatening complications

  • Venous stasis and sludging of blasts in small vessels cause hypoxia, hemorrhage, and infarction, most notably in the lung and CNS; this is a medical emergency requiring rapid intervention, such as leukophoresis, to decrease the leukocyte count

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Diagnosis

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  • Median hemoglobin value at diagnosis is 7 g/dL

  • Platelets usually number less than 50,000/μL

  • Frequently the absolute neutrophil count is under 1000/μL, although the total WBC count is over 100,000/μL in 25% of patients

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Treatment

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  • AML is less responsive to treatment than ALL and requires more intensive chemotherapy

  • AML protocols rely on intensive administration of anthracyclines, cytarabine, and etoposide for induction of remission

  • After remission is obtained,

    • Patients who have a matched sibling donor undergo allogeneic hematopoietic stem cell transplantation,

    • Patients without an appropriate related donor are treated with additional cycles of aggressive chemotherapy for a total of four to five cycles

  • When WBC cell count is > 100,000/μL or significant adenopathy or organomegaly is present

    • Maintain brisk urinary output

    • Serum levels of potassium, phosphorus, and uric acid should be monitored

  • Prompt initiation of broad-spectrum intravenous antibiotics when fever (temperature ≥ 38.3°C) or chills associated with and neutropenia develop during treatment

  • Patients nonimmune to varicella

    • Should receive varicella-zoster immune globulin within 72 hours after exposure

    • Should be given intravenous acyclovir for active infection

  • Pneumocystis prophylaxis must be administered during treatment and for several weeks afterward

  • Hyperleukocytosis (WBC > 100,000/μL)

    • Medical emergency

    • Requires rapid intervention such as leukophoresis to rapidly decrease the number of circulating blasts and thereby decrease hyperviscosity

  • Filgrastim (granulocyte colony-stimulating factor)

    • May be used to stimulate granulocyte recovery during the treatment of AML

    • Results in shorter periods of neutropenia and hospitalization

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