Hyperhaemolytic transfusion reaction in two β-thalassaemia major patients: The role of eculizumab.

delayed haemolytic transfusion reaction eculizumab haemoglobinopathies hyperhaemolysis thalassaemia

Journal

Journal of clinical pharmacy and therapeutics
ISSN: 1365-2710
Titre abrégé: J Clin Pharm Ther
Pays: England
ID NLM: 8704308

Informations de publication

Date de publication:
Mar 2022
Historique:
revised: 26 05 2021
received: 01 04 2021
accepted: 05 08 2021
pubmed: 17 8 2021
medline: 18 3 2022
entrez: 16 8 2021
Statut: ppublish

Résumé

Hyperhaemolytic transfusion reactions are rare life-threatening events predominantly affecting patients with haemoglobinopathies. We report two cases in β-thalassaemia major patients on chronic transfusion therapy and highlight the role of eculizumab in its management. Patient 1 presented with intravascular haemolysis on day 7 (D7) post-transfusion and responded to treatment with corticosteroids and intravenous immunoglobulin. However, patient 2 presented with severe symptomatic anaemia (D4 post-transfusion) unresponsive to the aforementioned measures. Eculizumab administration led to resolution of the hyperhaemolysis. We report the successful management of hyperhaemolysis with eculizumab in a β-thalassemia major patient.

Identifiants

pubmed: 34397109
doi: 10.1111/jcpt.13510
doi:

Substances chimiques

Antibodies, Monoclonal, Humanized 0
eculizumab A3ULP0F556

Types de publication

Case Reports

Langues

eng

Sous-ensembles de citation

IM

Pagination

411-414

Informations de copyright

© 2021 John Wiley & Sons Ltd.

Références

Panch SR, Montemayor-Garcia C, Klein HG. Hemolytic transfusion reactions. Longo DL, editor. N Engl J Med. 2019;381(2):150-162
Chonat S, Mener A, Verkerke H, Stowell SR. Role of complement in alloimmunization and hyperhemolysis. Curr Opin Hematol. 2020;27(6):406-414.
Pirenne F, Yazdanbakhsh K. How I safely transfuse patients with sickle-cell disease and manage delayed hemolytic transfusion reactions. Blood. 2018;131(25):2773-2781.
European Medicines Agency. Soliris (Eculizumab). https://www.ema.europa.eu/en/documents/overview/soliris-epar-medicine-overview_en.pdf. Accessed April 1, 2021.
Pirenne F. The cause and pathogenesis of hemolytic transfusion reactions in sickle-cell disease. Curr Opin Hematol. 2019;26(6):488-494.
Win N, Lucas S, Hebballi S, et al. Histopathological evidence for macrophage activation driving post-transfusion hyperhaemolysis syndrome. Br J Haematol. 2019;186(3):499-502.
Dubois EA, Cohen AF. Eculizumab. Br J Clin Pharmacol. 2009;68(3):318-319.
National Health System England. Clinical commissioning policy; rituximab and eculizumab for the prevention and management of delayed haemolytic transfusion reactions and hyperhaemolysis in patients with haemoglobinopathies. https://www.england.nhs.uk/wp-content/uploads/2020/09/1821_Rituximab_Eculizumab_Clinical_Commissioning_Policy.pdf. Accessed April 1, 2021.
Zanetti RC, Vasta LM, Romanelli K, Newton TC. Management of hyperhemolysis in β-thalassemia with multiple immunosuppressives, including complement blockade. J Pediatr Hematol Oncol. 2021. https://doi.org/10.1097/MPH.0000000000002059. Epub ahead of print.
Vagace JM, Cardesa R, Corbacho A, et al. Etiopathological mechanisms and clinical characteristics of hyperhemolysis syndrome in Spanish patients with thalassemia. Ann Hematol. 2016;95(9):1419-1427.

Auteurs

Despoina Pantelidou (D)

Thalassaemia Unit, AHEPA University Hospital, Thessaloniki, Greece.

Dimitrios Pilalas (D)

First Propedeutic Department of Internal Medicine, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece.

Stylianos Daios (S)

First Propedeutic Department of Internal Medicine, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece.

George Polychronopoulos (G)

First Propedeutic Department of Internal Medicine, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece.

Despoina Papadopoulou (D)

Thalassaemia Unit, AHEPA University Hospital, Thessaloniki, Greece.

Vassilios Perifanis (V)

First Propedeutic Department of Internal Medicine, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece.

Christos Savopoulos (C)

First Propedeutic Department of Internal Medicine, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece.

Georgia Kaiafa (G)

First Propedeutic Department of Internal Medicine, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece.

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