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Myelodysplasia

 

 

Down Syndrome (DS)

 

Down syndrome children have a significantly increased risk of leukemia

  • Age dependent:
    • More than 50 fold during the first 5 years of life
    • 10 fold from 5 to 29 years of age
    • After 30 risk same as individuals without DS
  • Half leukemias are myeloid

 

Transient Abnormal Myelopoiesis (TAM)

Also sometimes called transient leukemia

Clinical/morphological picture like congenital leukemia

10% of infants with DS have:

  • Increased leukocyte counts
  • Anemia
  • Thrombocytopenia

Blast cells have surface antigens of megakaryoblasts.

Clonal abnormalities in 35%.

Percentage of blasts in peripheral blood higher than bone marrow.

Leukemic skin infiltrates can occur and infants can develop life threatening liver disease.

Spontaneous remission in majority after about 1 - 3 months.

No chemotherapy generally given - but if serious liver disease then a short course of low dose cytarabine can be given.

 

Links:

National Cancer Institute: Children with Down syndrome

Myeloid Leukemia in DS

 

 

 

 

 

 

 

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