A severe transfusion‐dependent congenital dyserythropoietic anaemia presenting as hydrops fetalis

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 norma...

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Bibliographic Details
Published in:British journal of haematology 1997-03, Vol.96 (3), p.530-533
Main Authors: Cantù‐Rajnoldi, A., Zanella, A., Conter, U., Faccini, P., Soligo, D., Gornati, G., Vegni, C., Nicolini, U.
Format: Article
Language:English
Subjects:
Adult
Anemia, Dyserythropoietic, Congenital - blood
Anemia, Dyserythropoietic, Congenital - complications
Anemias. Hemoglobinopathies
Biological and medical sciences
Blood Transfusion
congenital dyserythropoietic anaemia
Diseases of red blood cells
Erythroblastosis, Fetal - blood
Erythroblastosis, Fetal - etiology
Female
fetal hydrops
Hematologic and hematopoietic diseases
Humans
Hydrops Fetalis - blood
Hydrops Fetalis - etiology
Infant, Newborn
Medical sciences
Microscopy, Electron
Pregnancy
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Summary: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.
ISSN:0007-1048
1365-2141
DOI:10.1046/j.1365-2141.1997.d01-2062.x