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

Author:

Remacha Angel F.1,Badell Isabel1,Pujol-Moix Núria1,Parra Juan1,Muñiz-Diaz Eduardo1,Ginovart Gemma1,Sardà M. Pilar1,Hernández Angel1,Moliner Elisenda1,Torrent Montserrat1

Affiliation:

1. From the Departments of Hematology, Pediatrics, Obstetrics, and Hemotherapy, Hospital Santa Creu i Sant Pau, Barcelona, Spain.

Abstract

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.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Reference5 articles.

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3. A severe transfusion-dependent congenital dyserythropoietic anaemia presenting as hydrops fetalis.;Cantù-Rajnoldi;Br J Haematol.,1997

4. Bone marrow transplantation in thalassemia: the experience of Pesaro.;Lucarelli;Ann N Y Acad Sci.,1998

5. Bone marrow transplantation in a case of severe, type II congenital dyserythropoietic anaemia (CDA II).;Iolascon;Bone Marrow Transplant.,2001

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