Open AccessPearson syndrome in a child transplanted for Diamond-Blackfan anemia
Author(s) -
Vedat Uygun,
Hayriye Daloğlu,
Seda Öztürkmen,
Gülsün Karasu,
Akif Yeşilipek
Publication year - 2021
Publication title -
archivos argentinos de pediatría
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.236
H-Index - 19
eISSN - 1668-3501
pISSN - 0325-0075
DOI - 10.5546/aap.2021.eng.e559
Subject(s) - diamond–blackfan anemia , medicine , anemia , pediatrics , gynecology , ribosome , rna , biochemistry , chemistry , gene
Pearson syndrome (PS), shares a number of overlapping features with Diamond-Blackfan anemia (DBA), including early onset of severe anemia, making differential diagnosis important. Differential diagnosis of DBA and PS is critical, since those with DBA may respond to treatment with steroids, may undergo remission, or may benefit from hematopoietic stem cell transplantation (HSCT). However, patients with PS have a different prognosis, with a very high risk of developing acidosis, metabolic problems, and pancreatic dysfunction, and a shorter life expectancy than those with DBA. Here we present a patient who underwent HSCT for DBA but was subsequently diagnosed with PS after developing some complications.