SUCCESSFUL MANAGEMENT OF CONGENITAL DYSERYTHROPOIETIC ANEMIA TYPE I WITH INTERFERON ALPHA IN A CHILD

Congenital dyserythropoietic anemia type I (CDA I) is a rare inherited hematological disorder characterized by macrocytic anemia and ineffective erythropoiesis with pathognomonic morphological features that include internuclear chromatin bridges, spongy heterochromatin, and invagination of the cytop...

Full description

Saved in:

Bibliographic Details
Published in:	Pediatric hematology and oncology Vol. 22; no. 4; pp. 265 - 270
Main Authors:	Yaral, Ne e, F g n, Tunç, Duru, Feride, Atilla, Pergin, Müftüo lu, Sevda F., Kaymaz, S. F.
Format:	Journal Article
Language:	English
Published:	England Informa UK Ltd 01-06-2005 Taylor & Francis
Subjects:	Anemia, Dyserythropoietic, Congenital - blood Anemia, Dyserythropoietic, Congenital - drug therapy congenital dyserythropoietic anemia type I Disease Management Erythrocytes - drug effects Erythrocytes - pathology Erythrocytes - ultrastructure Female Humans Infant Interferon-alpha - therapeutic use interferon-α Male Microscopy, Electron Treatment Outcome
Online Access:	Get full text
Tags:	Add Tag No Tags, Be the first to tag this record!

Description
Summary:	Congenital dyserythropoietic anemia type I (CDA I) is a rare inherited hematological disorder characterized by macrocytic anemia and ineffective erythropoiesis with pathognomonic morphological features that include internuclear chromatin bridges, spongy heterochromatin, and invagination of the cytoplasm into the nuclear area in erythroid precursors. Treatment of anemia with the usual hematinics is without effect and 15% of patients need chronic transfusions. Successful treatment of CDA I with interferon-α was noted. The authors report a patient with CDA I who had required transfusions every 2-3 months since the neonatal period and responded to recombinant interferon-α therapy with the findings of electron microscopic investigations.
Bibliography:	ObjectType-Case Study-2 SourceType-Scholarly Journals-1 ObjectType-Feature-4 content type line 23 ObjectType-Report-1 ObjectType-Article-3
ISSN:	0888-0018 1521-0669
DOI:	10.1080/08880010590935149