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Acute Myeloid Leukemia

 

 

 

Down Syndrome AML

 

Transient Leukemia in Down syndrome

Also called:

  • Transient Abnormal Hematopoiesis
  • Ineffective Regulation of Granulopoiesis
  • Transient Congenital Leukemia
  • Transient Leukemia-Like Disease
  • Transient Abnormal Myelopoiesis
  • Transient Leukemia-Like Blood Reaction
  • Pseudoleukemia

 

Overview:

  • Only seen in Down Syndrome patients in the newborn period
  • Uncontrolled proliferation of myeloblasts, frequently megakaryocytic origin
  • Spontaneous resolution typically in first 3 months of life
  • Incidence 10-20%

 

  • Presentation:
    • Hydrops
    • Hepatosplenomegaly
    • effusions (pleural, pericardial, peritoneal)
    • DIC
    • Renal failure
    • Respiratory failure

 

  • Lab Features:
    • Blood count:
      • Elevated white cell count with circulating myeloblasts
      • Normal platelets and hemoglobin usually
      • Normal neutrophils, basophilia
    • Bone Marrow:
      • Megakaryoblasts (CD41 and CD61 positive)
      • Lower blast percentage in marrow compared to peripheral blood

 

  • Treatment if:
    • Hyperviscosity signs and symptoms
    • Blast count >100
    • Organomegaly causing respiratory compromise
    • Congestive heart failure
    • Hydrops fetalis
    • Symptomatic hepatic or renal dysfunction
    • DIC with bleeding
  • Treat with leukapheresis or low dose cytarabine
  • Increased risk of AML in patients with history of TMD

 

AML in Down Syndrome

  • Leukemia in children with trisomy 21 mosaicism selectively involves the trisomic cells.
  • Trisomy 21 is the first hit in the multistep process leading to leukemia.
  • Incidence of leukemia peaks between birth and 4 years of age.
  • Develops in 1% of patients with DS.
  • 25% of patients with a morphologic diagnosis of RC, RAEB or RAEB-T have Down syndrome.
  • Bone marrow blasts <30% in most patients. Bone marrow often fibrotic and difficult to assess.
  • AML-M7 subtype often found in patients with Down Syndrome (otherwise rare) and often preceded by MDS.
  • Presents often with thrombocytopenia.
  • Blast cells have morphologic and antigenic features of megarokaryoblasts.
  • Usually responds well to standard therapy for AML (DS children have low risk of relapse but higher risk of therapy induced complications).

 

Link:

Children with Down Syndrome and leukemia at the National Cancer Institute

 

 

 

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