Hydrops fetalis-associated congenital dyserythropoietic anemia treated with intrauterine transfusions and bone marrow transplantation.

Abstract

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.

• Authors: Angel F Remacha, Isabel Badell, Núria Pujol-Moix, Juan Parra, Eduardo Muñiz-Diaz, Gemma Ginovart, M Pilar Sardà, Angel Hernández, Elisenda Moliner, Montserrat Torrent
• Journal: Blood (Blood) Vol. 100 Issue 1 Pg. 356-8 (Jul 01 2002) ISSN: 0006-4971 [Print] United States
• PMID: 12070051 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Anemia, Dyserythropoietic, Congenital (complications, therapy)
• Blood Transfusion, Intrauterine
• Bone Marrow Transplantation
• Family Health
• Female
• Fetal Diseases (therapy)
• Humans
• Hydrops Fetalis (diagnosis, etiology, therapy)
• Infant, Newborn
• Male
• Pregnancy
• Prenatal Diagnosis
• Transplantation, Homologous
• Treatment Outcome