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Snapshot
  • A 11-year-old girl with Down syndrome presents with a few weeks of low-grade fever. She is otherwise feeling well, with no signs of infection. Blood-work shows elevated WBCs, low neutrophil count, and anemia. Blood cultures are drawn and come back positive for Pseudomonas.
Introduction

  • Acute leukemia of lymphoid precursor cells that occurs in children
  • Crowding of bone marrow leads to bone marrow failure
  • Subtypes
    • B-cell ALL – 85% of all ALL
      • CD10+ and CD19/20+
      • TdT+ (marker of precursor T- or B-cell)
    • T-cell ALL
      • CD2+, CD8+, and CD3+
      • TdT+ (marker of precursor T- or B-cell)
      • commonly presents as mediastinal mass
        • infiltration of thymus
  • Epidemiology
    • < 15 years
    • most common type of cancer and leukemia in children
  • Associated conditions
    • Down syndrome in children > 5 years
      • leukemia in Down syndrome children < 5 years = AML
Presentation
  • Symptoms
    • most common symptom is fever
    • acute onset
      • recurrent infections
      • bleeding
      • fatigue
  • Physical exam
    • mediastinal mass from infiltration of thymus 
    • hepatosplenomegaly
    • lymphadenopathy   
Evaluation
  • Peripheral blood smear
    • ↑ lymphoblasts (high nuclei to cytoplasm ratio)
  • CBC reflects bone marrow failure
    • anemia
    • thrombocytopenia
    • ↓ mature WBCs
  • Bone marrow aspiration with cytogenetics
    • ↑ lymphoblasts
    • TdT+, a marker of pre-T and pre-B cells
    • T-cell ALL
      • CD2+
      • CD3+
    • B-cell AML
      • CD10+
      • CD19+
    • negative MPO (myeloperoxidase)
Differential Diagnosis
  • AML
  • B-cell lymphoma
  • Non-hodgkin lymphoma
Treatment
  • Chemotherapy
  • Prophylaxis to CNS (standard chemotherapy does not penetrate blood-brain barrier)
    • intrathecal chemotherapy
Prognosis, Prevention, and Complications
  • Prognosis
    • very responsive to therapy
  • Complications
    • likes to spread to CNS and testes
 

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