Hojo Satoshi, Tsukimori Kiyomi, Kitade Shoko, Nakanami Naoyuki, Hikino Shunji, Hara Toshiro, Wake Norio
Department of Obstetrics and Gynecology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Prenat Diagn. 2007 Jun;27(6):507-11. doi: 10.1002/pd.1718.
To determine relevant prenatal findings of transient abnormal myelopoiesis (TAM) that have important prognostic implications.
The prenatal and postnatal medical records of all cases with confirmed TAM associated with Down syndrome were reviewed retrospectively, with emphasis on prenatal sonographic findings, fetal blood analysis, neonatal outcomes, and causes of death.
From January 1992 to December 2005, seven cases were confirmed postnatally as having TAM associated with Down syndrome. Sonography demonstrated hydrops with hepatomegaly in four, and isolated hepatomegaly in two of these seven cases. There were no findings suggestive of cardiac failure in cases of hydrops. Fetal blood analysis revealed elevated liver enzyme levels in six cases and hypoalbuminemia in four cases. Comparison of sonographic findings with fetal blood findings demonstrated an association between hydrops and hypoalbuminemia. Four of the seven cases were fatal. All fatal cases were associated with hydrops and the main cause of death was coagulopathy due to liver failure, which may have resulted from infiltration of the liver by blast cells.
Fetal TAM is associated with hepatomegaly and elevated liver enzyme levels. The prenatal finding with prognostic implications is hydrops, which may result from hypoalbuminemia due to liver failure.
确定具有重要预后意义的短暂异常髓系造血（TAM）的相关产前检查结果。
回顾性分析所有确诊为与唐氏综合征相关的TAM病例的产前和产后病历，重点关注产前超声检查结果、胎儿血液分析、新生儿结局及死亡原因。
1992年1月至2005年12月，7例产后确诊为与唐氏综合征相关的TAM。超声检查显示，7例中有4例出现水肿伴肝肿大，2例仅有肝肿大。水肿病例中未发现提示心力衰竭的表现。胎儿血液分析显示，6例肝酶水平升高，4例低白蛋白血症。超声检查结果与胎儿血液检查结果比较显示，水肿与低白蛋白血症之间存在关联。7例中有4例死亡。所有死亡病例均与水肿有关，主要死亡原因是肝功能衰竭导致的凝血障碍，这可能是由于原始细胞浸润肝脏所致。
胎儿TAM与肝肿大及肝酶水平升高有关。具有预后意义的产前检查结果是水肿，这可能是肝功能衰竭导致低白蛋白血症所致。
