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Can progression from transient myeloid disease to AML be prevented in children with Down syndrome ?

Approximately 5-10% of children with Down syndrome (DS) are diagnosed with transient myeloproliferative disorder (TMD). Approximately 10-20% of these patients unfortunately decease within six months (early death), and another 20% progress to myeloid leukemia (ML-DS) within their first four years of life. In the Princess Máxima Center we research the progression of ML-DS, aiming at prevention.

 The TMD07 trial demonstrated that the progression to ML-DS cannot be prevented by treating patients with TMD with low-dose cytarabine. However, the prospective setup of the trial revealed the benefit of this well-tolerated treatment for symptomatic children: it significantly reduced deaths in the first six months compared with symptomatic patients in the historical control. This study helped in establishing clear recommendations for the management of children with TMD: patients that present with TMD-related clinical symptoms benefit from low-dose cytarabine treatment to prevent early death. This is now implemented as standard of care in the Princess Máxima Center.