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
