Incidence and treatment of potentially lethal diseases in transient XXXX of Down syndrome: Pediatric Oncology Group Study.

@entity1 @entity67888 @entity2592 @entity177 @entity1778 @entity1575 @entity204 @entity6961
Transient @entity1778 ( @entity67888 or transient @entity6961 ) occurs in approximately 10% of newborn @entity1 with Down syndrome. The disorder is characterized by the presence of megakaryoblasts in the peripheral blood; most cases resolve spontaneously within the first 3 months of life, and the @entity1 is well thereafter. However, there are cases in which a severe, potentially lethal form of disease develops, manifesting as @entity1575 or @entity177 . Hitherto, the incidence of these severe forms of the disease has not been reported. A prospective study of @entity67888 was conducted by the Pediatric Oncology Group (POG Study 9481) in which 48 @entity1 with @entity67888 were identified. Life-threatening disease occurred in nine @entity1 (19%); seven had @entity1575 and two had @entity177 . Five @entity1 died of the disease within the first 3 months of life, none of whom received antileukemic therapy. One @entity1 died on day 31 after receiving minimal therapy within 1 day of @entity204 . Three @entity1 received low-dose @entity2592 ( @entity2592 ) (0.4-1.5 mg/kg every 12 hours for 5 or 7 days). In all these @entity1 , the disease resolved. It is concluded that potentially lethal disease is relatively common in @entity67888 , and the available evidence suggests that these diseases are responsive to low-dose @entity2592 therapy.
