Li-Thiao-Te V, Bourges-Petit E, Capiod J C, Horle B, Micheli J, Morin G, Maingourd Y, Pautard B
Service d'hématologie-oncologie pédiatrique, CHU Nord, place Victor-Pauchet, 80054 Amiens cedex, France.
Arch Pediatr. 2008 Jan;15(1):33-6. doi: 10.1016/j.arcped.2007.08.023. Epub 2007 Dec 26.
Neonates with Down's syndrome have an increased risk for congenital leukaemia, particularly acute megakaryoblastic leukaemia (FAB, M7) which most often resolves spontaneously and is called transient leukaemia. It can be observed in non-constitutional trisomy 21 infants then presenting trisomy 21 on blasts cells.
We report a transient leukaemia with an isolated pericardial effusion in a phenotypically normal neonate. Trisomy 21 was found on blasts cells. Complete remission remains after 32 months.
Congenital leukaemias, with trisomy 21 on blasts cells have a good prognosis that justifies observation before using chemotherapy.
唐氏综合征新生儿患先天性白血病的风险增加,尤其是急性巨核细胞白血病(FAB,M7),这种白血病大多会自发缓解,称为暂时性白血病。在非染色体三体21婴儿中可观察到,其原始细胞呈现三体21。
我们报告了一例表型正常的新生儿出现孤立性心包积液的暂时性白血病。原始细胞发现有三体21。32个月后仍处于完全缓解状态。
原始细胞有三体21的先天性白血病预后良好,这证明在使用化疗之前进行观察是合理的。