Diamond-Blackfan anaemia with iron overload: A serious issue

Paola Quarello; Ugo Ramenghi; Franca Fagioli

doi:10.1111/bjh.18393

Diamond-Blackfan anaemia with iron overload: A serious issue

Br J Haematol. 2022 Oct;199(2):171-172. doi: 10.1111/bjh.18393. Epub 2022 Aug 5.

Authors

Paola Quarello¹, Ugo Ramenghi², Franca Fagioli¹

Affiliations

¹ Pediatric Onco-Hematology, Stem Cell Transplantation and Cellular Therapy Division, Regina Margherita Children's Hospital, Azienda Ospedaliera-Universitaria Città della Salute e della Scienza, University of Torino, Torino, Italy.
² Department of Pediatric and Public Health Sciences, University of Turin, Turin, Italy.

PMID: 35929529
DOI: 10.1111/bjh.18393

Abstract

Transfusion-dependent Diamond-Blackfan anaemia (DBA) patients rapidly develop iron overload and frequently experience cardiac complications. The report by Lecornec and colleagues offers useful details on indications and the management of deferiprone, a highly efficient chelator in removing excess cardiac iron but associated with a high risk of agranulocytosis in DBA patients. Commentary on: Lecornec et al. Agranulocytosis in patients with Diamond-Blackfan anaemia (DBA) treated with deferiprone for post-transfusion iron overload: A retrospective study of the French DBA cohort. British Journal of Haematology 2022;199:285-288.

MeSH terms

Agranulocytosis*
Anemia, Diamond-Blackfan* / complications
Anemia, Diamond-Blackfan* / therapy
Chelating Agents
Deferiprone / therapeutic use
Humans
Iron / therapeutic use
Iron Overload* / complications
Retrospective Studies

Substances

Chelating Agents
Deferiprone
Iron