Submitted December 28, 2001
Accepted March 1, 2002
Hydrops fetalis associated congenital dyserythropoietic anemia (CDA) treated with intrauterine transfusions and bone marrow transplantation
Angel F Remacha*, Isabel Badell, Núria Pujol-Moix, Juan Parra, Eduardo Muniz-Diaz, Gemma Ginovart, M.Pilar Sardà, Angel Hernandez, Elisenda Moliner, and Montserrat Torrent
Hematology, Hospital Sant Pau, Barcelona, Spain
Pediatrics, Hospital Sant Pau, Barcelona, Spain
Obstetrics, Hospital Sant Pau, Barcelona, Spain
Hemotherapy, Hospital Sant Pau, Barcelona, Spain
* Corresponding author; email: aremacha{at}hsp.santpau.es.
Hydrops fetalis is rarely caused by congenital dyserythropoietic anemia (CDA). We report a patient with hydrops fetalis due to severe anemia. This patient needed intrauterine transfusions from 21 weeks of gestation until birth. The hematological study showed an atypical CDA (hydrops fetalis associated CDA) characterised by features resembling CDA type II, but negative acidified serum lysis test (HEMPAS negative). The patient was regularly transfused for a year after which an allogeneic bone marrow transplantation (BMT) from an HLA-identical sibling was successfully carried out. His actual Hb is 127 g/l and he has not received transfusions for more than a year. In conclusion, intrauterine transfusions and BMT could cure an otherwise lethal atypical CDA.
