Cantù-Rajnoldi A, Zanella A, Conter U, Faccini P, Soligo D, Gornati G, Vegni C, Nicolini U
Anatomia ed Istoligia Patologica, Istituti Clinici di Perfezionamento, Milano, Italia.
Br J Haematol. 1997 Mar;96(3):530-3. doi: 10.1046/j.1365-2141.1997.d01-2062.x.
We describe a case of congenital dyserythropoietic anaemia (CDA) presenting at week 20 of pregnancy with hydrops fetalis and very severe anaemia. Fetal blood examination showed marked erythroblastosis with morphologic alterations while the basic haematological and biochemical tests were in the normal range. The fetus received intravascular red blood cells transfusion at 20 and 25 weeks of gestation. After a caesarean section at week 33 the child became transfusion dependent. Haematological investigations demonstrated that the child is affected by a CDA-like picture which, although morphologically similar to CDA II, is Ham test negative and does not show the typical membrane proteins alterations.
我们描述了一例先天性红细胞生成异常性贫血（CDA）病例，该病例在妊娠20周时出现胎儿水肿和非常严重的贫血。胎儿血液检查显示有明显的成红细胞增多症并伴有形态学改变，而基本血液学和生化检查结果在正常范围内。胎儿在妊娠20周和25周时接受了血管内红细胞输血。在33周剖宫产术后，该患儿变得依赖输血。血液学检查表明，该患儿呈现出类似CDA的表现，尽管在形态学上与CDA II相似，但酸化血清溶血试验（Ham试验）为阴性，且未显示典型的膜蛋白改变。
