Blood, 1 July 2002, Vol. 100, No. 1, pp. 356-358
BRIEF REPORT
Hydrops fetalis-associated congenital dyserythropoietic anemia
treated with intrauterine transfusions and bone marrow
transplantation
Angel F. Remacha,
Isabel Badell,
Núria Pujol-Moix,
Juan Parra,
Eduardo Muñiz-Diaz,
Gemma Ginovart,
M. Pilar Sardà,
Angel Hernández,
Elisenda Moliner, and
Montserrat Torrent
From the Departments of Hematology, Pediatrics,
Obstetrics, and Hemotherapy, Hospital Santa Creu i Sant Pau, Barcelona,
Spain.
Hydrops fetalis is rarely caused by congenital dyserythropoietic
anemia (CDA). We report a patient with hydrops fetalis as a result of
severe anemia. This patient needed intrauterine transfusions from 21 weeks of gestation until birth. The hematologic study showed an
atypical CDA (hydrops fetalis-associated CDA) characterized 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
hemoglobin 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.
