Successful treatment with plasma exchange in life-threatening hyperhemolytic syndrome unrelated to sickle cell disease.
eculizumab
hyperhemolytic syndrome
plasma exchange
Journal
Transfusion
ISSN: 1537-2995
Titre abrégé: Transfusion
Pays: United States
ID NLM: 0417360
Informations de publication
Date de publication:
05 2023
05 2023
Historique:
revised:
02
01
2023
received:
12
10
2022
accepted:
07
02
2023
medline:
11
5
2023
pubmed:
4
3
2023
entrez:
3
3
2023
Statut:
ppublish
Résumé
Hyperhemolytic syndrome (HHS) is a severe form of delayed transfusion reaction primarily described in sickle cell anemia patients which is characterized by a hemoglobin decrease to pre-transfusion levels or lower, often with reticulocytopenia and no evidence of auto- or allo-antibodies. We present two cases of severe HHS in patients without sickle cell anemia refractory to treatment with steroids, immunoglobulins, and rituximab. In one case, temporary relief was achieved with eculizumab. In both cases, plasma exchange resulted in a profound and immediate response allowing for splenectomy and resolution of hemolysis. We discuss the pathophysiology of HHS, its presentation and treatment and expand on the possible role of plasma exchange in this setting.
Substances chimiques
Steroids
0
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
1100-1106Informations de copyright
© 2023 The Authors. Transfusion published by Wiley Periodicals LLC on behalf of AABB.
Références
Diamond WJ, Brown FL, Bitterman P, Klein HG, Davey RJ, Winslow RM. Delayed hemolytic transfusion reaction presenting as sickle-cell crisis. Ann Intern Med. 1980;93(2):231-4.
Gardner K, Hoppe C, Mijovic A, Thein SL. How we treat delayed haemolytic transfusion reactions in patients with sickle cell disease. Br J Haematol. 2015;170(6):745-56.
Win N, Doughty H, Telfer P, Wild BJ, Pearson TC. Hyperhemolytic transfusion reaction in sickle cell disease. Transfusion (Paris). 2001;41(3):323-8.
Sirchia G, Morelati F, Rebulla P. The sickle cell hemolytic transfusion reaction syndrome. Transfusion (Paris). 1997;37(10):1098-9. author reply 1100-1102.
Yan M, Callum J, Lin Y. Hyperhemolysis associated with marginal zone lymphoma. Leuk Lymphoma. 2015;56(3):829-31.
Treleaven JG, Win N. Hyperhaemolysis syndrome in a patient with myelofibrosis. Hematology (Amsterdam, Netherlands). 2004;9(2):147-9.
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;43(8):e1145-7.
Darabi K, Dzik S. Hyperhemolysis syndrome in anemia of chronic disease. Transfusion (Paris). 2005;45(12):1930-3.
Gupta S, Fenves A, Nance ST, Sykes DB, Dzik WS. Hyperhemolysis syndrome in a patient without a hemoglobinopathy, unresponsive to treatment with eculizumab. Transfusion (Paris). 2015;55(3):623-8.
Danaee A, Inusa B, Howard J, Robinson S. Hyperhemolysis in patients with hemoglobinopathies: a single-center experience and review of the literature. Transfus Med Rev. 2015;29(4):220-30.
King KE, Shirey RS, Lankiewicz MW, Young-Ramsaran J, Ness PM. Delayed hemolytic transfusion reactions in sickle cell disease: simultaneous destruction of recipients' red cells. Transfusion (Paris). 1997;37(4):376-81.
Zimring JC, Cadwell CM, Chadwick TE, Spitalnik SL, Schirmer DA, Wu T, et al. Nonhemolytic antigen loss from red blood cells requires cooperative binding of multiple antibodies recognizing different epitopes. Blood. 2007;110(6):2201-8.
Chadebech P, Habibi A, Nzouakou R, Bachir D, Meunier-Costes N, Bonin P, et al. Delayed hemolytic transfusion reaction in sickle cell disease patients: evidence of an emerging syndrome with suicidal red blood cell death. Transfusion (Paris). 2009;49(9):1785-92.
Petz LD, Calhoun L, Shulman IA, Johnson C, Herron RM. The sickle cell hemolytic transfusion reaction syndrome. Transfusion (Paris). 1997;37(4):382-92.
Win N. Hyperhemolysis syndrome in sickle cell disease. Expert Rev Hematol. 2009;2(2):111-5.
Mwesigwa S, Moulds JM, Chen A, Flanagan J, Sheehan VA, George A, et al. Whole-exome sequencing of sickle cell disease patients with hyperhemolysis syndrome suggests a role for rare variation in disease predisposition: WES in SCD patients with HHS. Transfusion (Paris). 2018;58(3):726-35.
Aline Floch, Alexandre Morel, Fabian Zanchetta-Balint, Catherine Cordonnier-Jourdin, Slimane Allali, Maximilien Grall, et al. Anti-C5 antibody treatment for delayed hemolytic transfusion reactions in sickle cell disease. Haematologica 2020 2;105(11):2694-7.
Menakuru SR, Priscu A, Dhillon V, Salih A. Acute hyperhemolysis syndrome in a patient with known sickle cell anemia refractory to steroids and IVIG treated with tocilizumab and erythropoietin: a case report and review of literature. Hematol Rep 2022 21;14(3):235-9.
Garratty G. The James Blundell award lecture 2007: do we really understand immune red cell destruction? Transfus Med Oxf Engl. 2008;18(6):321-34.
Unnikrishnan A, Pelletier JPR, Bari S, Zumberg M, Shahmohamadi A, Spiess BD, et al. Anti-N and anti-Doa immunoglobulin G alloantibody-mediated delayed hemolytic transfusion reaction with hyperhemolysis in sickle cell disease treated with eculizumab and HBOC-201: case report and review of the literature. Transfusion (Paris). 2019 Feb 15;59:1907-10.
Gavriilaki E, Mainou M, Christodoulou I, Koravou EE, Paleta A, Touloumenidou T, et al. In vitro evidence of complement activation in patients with sickle cell disease. Haematologica. 2017;102(12):e481-2.
Chonat S, Mener A, Verkerke H, Stowell SR. Role of complement in alloimmunization and hyperhemolysis. Curr Opin Hematol. 2020;27(6):406-14.
Patel I, Odak M, Douedi S, Alshami A, Upadhyaya VD, Hossain M, et al. Eculizumab as a treatment for hyper-Haemolytic and aplastic crisis in sickle cell disease. Eur J Case Rep Intern Med. 2021;8(10):002824.
Bank I, Ermens A a M, Linden JMVD, Brand A. A life-threatening episode of treatment-resistant haemolysis in a pregnant patient with dyserythropoietic anaemia (CDA) type I. Transfus Med. 2012;22(2):145-7.
Grainger JD, Makar Y, McManus A, Wynn R. Refractory hyperhaemolysis in a patient with β-thalassaemia major. Transfus Med. 2001;11(1):55-7.
Uhlmann EJ, Shenoy S, Goodnough LT. Successful treatment of recurrent hyperhemolysis syndrome with immunosuppression and plasma-to-red blood cell exchange transfusion. Transfusion (Paris). 2014;54(2):384-8.
Kalyanaraman M, Heidemann SM, Sarnaik AP, Meert KL, Sarnaik SA. Anti-s antibody-associated delayed hemolytic transfusion reaction in patients with sickle cell anemia. J Pediatr Hematol Oncol. 1999;21(1):70-3.
Bartolucci P, Noizat Pirenne F, Habibi A. Management of hyperhaemolysis after a transfusion in sickle-cell patients. ISBT Sci Ser. 2016;11(S1):196-200.
McLeod BC. An approach to evidence-based therapeutic apheresis. J Clin Apheresis. 2002;17(3):124-32.
Padmanabhan A, Connelly-Smith L, Aqui N, Balogun RA, Klingel R, Meyer E, et al. Guidelines on the use of therapeutic apheresis in clinical practice - evidence-based approach from the writing Committee of the American Society for apheresis: the eighth special issue. J Clin Apheresis. 2019;34(3):171-354.