Transfusion burden in early childhood plays an important role in iron overload in Diamond-Blackfan anaemia

Jonathan R A de Wilde; Birgit van Dooijeweert; Annelies J van Vuren; Elise J Huisman; Frans J Smiers; Arian van der Veer; Richard van Wijk; Wouter W van Solinge; Edward E S Nieuwenhuis; Eduard J van Beers; Marije Bartels

doi:10.1002/jha2.524

Transfusion burden in early childhood plays an important role in iron overload in Diamond-Blackfan anaemia

Jonathan R A de Wilde, Birgit van Dooijeweert, Annelies J van Vuren, Elise J Huisman, Frans J Smiers, Arian van der Veer, Richard van Wijk, Wouter W van Solinge, Edward E S Nieuwenhuis, Eduard J van Beers, Marije Bartels^*

^*Corresponding author for this work

Research output: Contribution to journal › Article › Academic › peer-review

Abstract

In Diamond-Blackfan anaemia (DBA), iron overload (IO) is common in transfusion-dependent patients, yet has also been reported in non-transfusion-dependent patients. We explored the incidence of IO in transfusion-dependent and non-transfusion-dependent DBA patients. We observed hepatic IO in 65% of patients analysed with MRI, including three patients that were only treated with transfusions in the past. Whereas overall ferritin levels and liver iron content correlated, ferritin levels did not reflect total body iron adequately. Our data suggest that transfusion burden in the past plays an important role in IO in DBA, and should be taken into account during follow up.

Original language	English
Pages (from-to)	1300-1304
Number of pages	5
Journal	eJHaem
Volume	3
Issue number	4
DOIs	https://doi.org/10.1002/jha2.524
Publication status	Published - Nov 2022

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de Wilde, J. R. A., van Dooijeweert, B., van Vuren, A. J., Huisman, E. J., Smiers, F. J., van der Veer, A., van Wijk, R., van Solinge, W. W., Nieuwenhuis, E. E. S., van Beers, E. J., & Bartels, M. (2022). Transfusion burden in early childhood plays an important role in iron overload in Diamond-Blackfan anaemia. eJHaem, 3(4), 1300-1304. https://doi.org/10.1002/jha2.524

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title = "Transfusion burden in early childhood plays an important role in iron overload in Diamond-Blackfan anaemia",

abstract = "In Diamond-Blackfan anaemia (DBA), iron overload (IO) is common in transfusion-dependent patients, yet has also been reported in non-transfusion-dependent patients. We explored the incidence of IO in transfusion-dependent and non-transfusion-dependent DBA patients. We observed hepatic IO in 65% of patients analysed with MRI, including three patients that were only treated with transfusions in the past. Whereas overall ferritin levels and liver iron content correlated, ferritin levels did not reflect total body iron adequately. Our data suggest that transfusion burden in the past plays an important role in IO in DBA, and should be taken into account during follow up.",

author = "{de Wilde}, {Jonathan R A} and {van Dooijeweert}, Birgit and {van Vuren}, {Annelies J} and Huisman, {Elise J} and Smiers, {Frans J} and {van der Veer}, Arian and {van Wijk}, Richard and {van Solinge}, {Wouter W} and Nieuwenhuis, {Edward E S} and {van Beers}, {Eduard J} and Marije Bartels",

note = "{\textcopyright} 2022 The Authors. eJHaem published by British Society for Haematology and John Wiley & Sons Ltd.",

year = "2022",

month = nov,

doi = "10.1002/jha2.524",

language = "English",

volume = "3",

pages = "1300--1304",

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issn = "2688-6146",

publisher = "John Wiley & Sons Inc.",

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T1 - Transfusion burden in early childhood plays an important role in iron overload in Diamond-Blackfan anaemia

AU - de Wilde, Jonathan R A

AU - van Dooijeweert, Birgit

AU - van Vuren, Annelies J

AU - Huisman, Elise J

AU - Smiers, Frans J

AU - van der Veer, Arian

AU - van Wijk, Richard

AU - van Solinge, Wouter W

AU - Nieuwenhuis, Edward E S

AU - van Beers, Eduard J

AU - Bartels, Marije

PY - 2022/11

Y1 - 2022/11

N2 - In Diamond-Blackfan anaemia (DBA), iron overload (IO) is common in transfusion-dependent patients, yet has also been reported in non-transfusion-dependent patients. We explored the incidence of IO in transfusion-dependent and non-transfusion-dependent DBA patients. We observed hepatic IO in 65% of patients analysed with MRI, including three patients that were only treated with transfusions in the past. Whereas overall ferritin levels and liver iron content correlated, ferritin levels did not reflect total body iron adequately. Our data suggest that transfusion burden in the past plays an important role in IO in DBA, and should be taken into account during follow up.

AB - In Diamond-Blackfan anaemia (DBA), iron overload (IO) is common in transfusion-dependent patients, yet has also been reported in non-transfusion-dependent patients. We explored the incidence of IO in transfusion-dependent and non-transfusion-dependent DBA patients. We observed hepatic IO in 65% of patients analysed with MRI, including three patients that were only treated with transfusions in the past. Whereas overall ferritin levels and liver iron content correlated, ferritin levels did not reflect total body iron adequately. Our data suggest that transfusion burden in the past plays an important role in IO in DBA, and should be taken into account during follow up.

U2 - 10.1002/jha2.524

DO - 10.1002/jha2.524

M3 - Article

C2 - 36467824

SN - 2688-6146

VL - 3

SP - 1300

EP - 1304

JO - eJHaem

JF - eJHaem

IS - 4

ER -

Transfusion burden in early childhood plays an important role in iron overload in Diamond-Blackfan anaemia

Abstract

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