Optimizing transfusion therapy for survivors of Haemoglobin Bart's hydrops fetalis syndrome: Defining the targets for haemoglobin‐H fraction and 'functional' haemoglobin level

Autor: Ali Amid, Nick Barrowman, Isaac Odame, Melanie Kirby‐Allen
Rok vydání: 2022
Předmět:
Zdroj: British Journal of Haematology. 197:373-376
ISSN: 1365-2141
0007-1048
DOI: 10.1111/bjh.18077
Popis: Owing to the unique pathophysiology of anaemia in haemoglobin Bart's hydrops fetalis (HBHF), a transfusion strategy based on beta-thalassemia guidelines is suboptimal for chronically transfused HBHF patients. A more aggressive transfusion aimed at reducing the proportion of non-functional HbH and improving the "functional" haemoglobin (f-Hb) can lead to reduced haemolysis and improved tissue oxygenation. However, the optimal transfusion targets for these parameters are not yet defined. In this retrospective, longitudinal study on four chronically transfused patients with HBHF, we used receiver operating characteristic curves to find a pre-transfusion f-Hb of 106 g/l and a HbH of 16.1% to be the optimal thresholds to achieve a normal soluble transferrin receptor and lactate dehydrogenase, respectively.
Databáze: OpenAIRE
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