• Bone marrow aspirate must contain >30% myeloblasts.
• Immunophenotyping
  • Blasts positive for myeloid antigens
  • CD11, CD13, CD14, CD15, CD33, CD34
  • CD41 and CD42 (megakaryocytic)
• Morphology
  • Large blasts with low nuclear/cytoplasmic ratio
  • Multiple nucleoli and cytoplasmic granules
• Cytochemistry
  • Blasts are positive for myeloperoxidase and sudan black and usually negative for periodic acid. Schiff (PAS) and terminal deoxynuleotide transferase (TdT)

• 15% to 20% of childhood acute leukemia is AML
• Leukemia in first 4 weeks of life is usually AML
• Incidence slightly increased in teens
• Ratio of AML to ALL throughout childhood is 1:4
• Boys and girls equally affected

• Bleeding
• Disseminated intravascular coagulation (DIC) occurs in some varieties of AML
• Infection
  • 40% patients are febrile at diagnosis
• Leukostasis
  • Intravascular clumping of blasts causing hypoxia, infarction, and hemorrhage.
  • Usually with white blood count (WBC) >200,000/mm³
• Tumor lysis syndrome and renal failure due to hyperuricemia
• Failure to induce remission in 15% to 20% of pediatric cases

• 85% achieve remission with intensive chemotherapy
• About 30% to 40% achieve long-term survival (more than 5 years after diagnosis)

• Myeloid blast crisis of chronic myeloid leukemia (Philadelphia chromosome positive)
• Acute lymphoblastic leukemia
• Leukemoid reaction
• Exaggerted leukocytosis

Question: Fever (40% cases)
Significance: Infection

Question: Pallor (25% cases)
Significance: Anemia

Question: Anorexia and weight loss (22% cases)
Significance: Systemic symptoms of malignancy

Question: Fatigue (19% cases)
Significance: Anemia

Question: Bleeding (33% cases)
Significance: Cutaneous and mucosal thrombocytopenia

Question: Bone pain (18% cases)
Significance: Bone marrow infiltration

Question: Headache/vomiting
Significance: Meningeal leukemia, intracranial bleeding

Question: Repeated infections
Significance: Persistant neutropenia due to leukemia

Finding: Pallor
Significance: Anemia

Finding: Petechiae, purpura, bleeding
Significance: Thrombocytopenia

Finding: Purple skin lesions
Significance: Leukemic infiltrate (leukemia cutis)

Finding: Swollen gingiva
Significance: Leukemic infiltration

Finding: Mass lesions
Significance: Chloromas (leukemic tumors)

Finding: Lymphadenopathy (<25% cases) or Hepatosplenomegaly (50% cases)
Significance: Leukemic infiltration

Finding: Testicular enlargement (rare)
Significance: Testicular infiltration

Test: CBC
Significance: Anemia, thrombocytopenia, WBC count usually >10000/mm³

Test: Peripheral smear
Significance: Myeloblasts may be seen.

Test: Bone Marrow aspirate
Significance: Diagnostic; >30% myeloblasts; confirmed by immunophenotyping and cytochemistry

Test: PT, PTT and fibrin split products (FSP) increased. Fibrinogen may be decreased
Significance: Coagulopathy, disseminated vascular coagulation

Test: Hyperkalemia, hypocalcemia, hyperphosphatemia and hyperuricemia
Significance: Presence of tumor lysis

Test: Cerebrospinal fluid cytology
Significance: >5 WBC/mm³ suggestive of central nervous system (CNS) disease; 5% to 15% of cases have CNS disease at diagnosis.

• WBC count >100,000/mm³
• Monosomy 7
• Secondary AML or prior myelodysplastic syndrome

• Most effective drugs for remission induction in AML.
• Anthracyclines
  • Doxorubicin
  • Daunomycin
• Cytosine Arabinoside (ARA-C) with or without 6-thioguanine (6TG)
• VP16 and Dexamethasone added in some regimen
• High rate of remission induction with Trans retinoic acid in Acute promyelocytic leukemia
• Consolidation therapy usually with ARA-C and L-asparaginase
• Intrathecal ARA-C for CNS prophylaxis
• Maintenance with VP16, ARA-C, 6TG, Daunomycin, Dexamethasone
• Duration of therapy is usually 6–9 months
• Allogeneic bone marrow transplant may be the best treatment for AML in first remission

• Hydration, alkalization, and Allopurinol during induction
• Blood product support
  • Avoid products from family members if possibility of allogeneic bone marrow transplant
• Broad spectrum antibiotics and antifungal therapy for fever and neutropenia
• Prophylactic trimethoprim/sulphamethoxyzole for Pneumocystis
• Nystatin/fluconozole for fungal prophylaxis
• Heparin may be needed for treatment of DIC.

Q: Is an indwelling line required for therapy?
A: Always.

Q: Are repeated hospitalizations likely?
A: Repeated hospitalizations are needed for chemotherapy and infectious complications.

Q: Can the child go to school?
A: May be able to go intermittently during therapy.

Ebb DH, Weinstein HJ. Diagnosis and treatment of childhood AML. Pediatr Clin North Am 1997;44(4):847–862.

Hurwitz CA, Mounce KG, Grier HE. Treatment of patients with AML: review of clinical trials of past decade. J Pediatr Hematol Oncol 1995;17(3):185–197.

Kersey JH. Fifty years of studies of biology and therapy of chilhood leukemia. Blood 1997;90(11):4243–4251.