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Updated: Oct 12 2017

Acute Lymphoblastic Leukemia (ALL)

Snapshot
  • A 7-year-old boy presents with low-grade fever and fatigue. He is notably pale. On physical exam, he has bilateral cervical lymphadenopathy and a mediastinal mass. Blood smear shows increased lymphoblasts. He is referred for a bone marrow biopsy.
Introduction

  • Acute leukemia of lymphoid precursor cells that occurs in children
  • Subtypes
    • B-cell ALL – 85% of all ALL
      • t(12;21) most common in children
      • t(9;22) most common in adults
        • same translocation seen in CML
      • CD10+ and CD19/20+
      • TdT+ (marker of precursor T- and B-cells)
    • T-cell ALL
      • presents in teenagers
      • CD2-8+ (especially CD3+)
      • TdT+ (marker of precursor T- and B-cells)
      • most common presentation is mediastinal mass 
        • infiltration of thymus
  • Epidemiology 
    • < 15 years
    • most common type of cancer and leukemia in children
    • most cases with no identifiable risk factor
  • 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
  • Best initial test - peripheral blood smear
    • lymphoblasts
  • Most accurate test
    • bone marrow biopsy with cytogenetics
      • ↑ lymphoblasts (> 25%)
      • TdT+, a marker of pre-T and pre-B cells
      • T-cell ALL
        • CD3+
      • B-cell ALL
        • CD10+
        • CD19+
      • negative MPO (myeloperoxidase)
  • CBC reflects bone marrow crowding by neoplasm
    • anemia
    • thrombocytopenia
    • ↓ mature WBCs
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
    • good prognosis in t(12;21)
    • worse prognosis in t(9;22)
  • Complications
    • likes to spread to CNS and testes
    • DIC
Question
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