Eculizumab as a Treatment for Hyper-Haemolytic and Aplastic Crisis in Sickle Cell Disease.
Sickle cell disease
aplastic anemia
eculizumab
hyper-hemolysis
Journal
European journal of case reports in internal medicine
ISSN: 2284-2594
Titre abrégé: Eur J Case Rep Intern Med
Pays: Italy
ID NLM: 101648453
Informations de publication
Date de publication:
2021
2021
Historique:
received:
08
08
2021
accepted:
26
08
2021
entrez:
18
11
2021
pubmed:
19
11
2021
medline:
19
11
2021
Statut:
epublish
Résumé
Patients with sickle cell disease can experience various crises including sequestration crisis, haemolytic crisis and aplastic crisis. Due to alloantibody formation, transfusion alloantibodies can cause a haemolytic crisis. Treatment involves avoiding packed red blood cell transfusions, as well as intravenous immunoglobulin, steroids and eculizumab to decrease the chances of haemolysis. We report the case of a 42-year-old man who was found to have worsening anaemia after packed red blood cell transfusion with evidence suggestive of haemolytic crisis. Due to reticulocytopenia, aplastic crisis was also suspected and later confirmed via parvovirus IgG and IgM titres. The patient did not improve with steroid and intravenous immunoglobulin therapy and was treated with eculizumab as a salvage therapy. Concurrent hyper-haemolytic crisis and aplastic crisis should be suspected in patients with features of haemolysis and reticulocytopenia. Prompt recognition and treatment with eculizumab are paramount in those who fail steroid and intravenous immunoglobulin treatment. Treatment of hyper-haemolytic and aplastic crisis in sickle cell disease with eculizumab offers therapeutic benefit.A high index of suspicion for hyper-haemolytic crisis and aplastic crisis should be maintained in those with haemolytic features as well as reticulocytopenia in the setting of sickle cell disease.
Sections du résumé
BACKGROUND
BACKGROUND
Patients with sickle cell disease can experience various crises including sequestration crisis, haemolytic crisis and aplastic crisis. Due to alloantibody formation, transfusion alloantibodies can cause a haemolytic crisis. Treatment involves avoiding packed red blood cell transfusions, as well as intravenous immunoglobulin, steroids and eculizumab to decrease the chances of haemolysis.
CASE DESCRIPTION
METHODS
We report the case of a 42-year-old man who was found to have worsening anaemia after packed red blood cell transfusion with evidence suggestive of haemolytic crisis. Due to reticulocytopenia, aplastic crisis was also suspected and later confirmed via parvovirus IgG and IgM titres. The patient did not improve with steroid and intravenous immunoglobulin therapy and was treated with eculizumab as a salvage therapy.
CONCLUSION
CONCLUSIONS
Concurrent hyper-haemolytic crisis and aplastic crisis should be suspected in patients with features of haemolysis and reticulocytopenia. Prompt recognition and treatment with eculizumab are paramount in those who fail steroid and intravenous immunoglobulin treatment.
LEARNING POINTS
CONCLUSIONS
Treatment of hyper-haemolytic and aplastic crisis in sickle cell disease with eculizumab offers therapeutic benefit.A high index of suspicion for hyper-haemolytic crisis and aplastic crisis should be maintained in those with haemolytic features as well as reticulocytopenia in the setting of sickle cell disease.
Identifiants
pubmed: 34790624
doi: 10.12890/2021_002824
pii: 2824-1-24741-1-10-20211005
pmc: PMC8592653
doi:
Types de publication
Journal Article
Langues
eng
Pagination
002824Informations de copyright
© EFIM 2021.
Déclaration de conflit d'intérêts
Conflicts of Interests: The authors declare there are no competing interests.
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