Protocol for extramedullary hematopoiesis in patients with transfusion-dependent β-thalassemia (TDT): A systematic review.
beta‐thalassemia major
extramedullary hematopoiesis
non‐medullary hematopoiesis
transfusion‐dependent thalassemia
Journal
Health science reports
ISSN: 2398-8835
Titre abrégé: Health Sci Rep
Pays: United States
ID NLM: 101728855
Informations de publication
Date de publication:
Dec 2021
Dec 2021
Historique:
received:
12
05
2021
revised:
18
09
2021
accepted:
27
09
2021
entrez:
11
11
2021
pubmed:
12
11
2021
medline:
12
11
2021
Statut:
epublish
Résumé
Thalassemia is one of the most common hemoglobinopathies, with around 5% of the world's population expected to have some degree and type of thalassemia. Beta thalassemia (BT) occurs due to a deficient production of the beta-globin chain of hemoglobin. Extramedullary hematopoiesis (EMH) is one of the complications of BT, mainly observed in minor/intermedia subtypes. EMH is the production of blood cells outside the marrow as a compensatory response to longstanding hypoxia. Due to chronic transfusions, it is not expected in patients with beta-thalassemia major (BTM). However, there are increasingly reported cases of EMH in BTM. The incidence of EMH in BTM is thought to be <1%. However, it seems that the true incidence is much higher than expected. This review aims to pool the available data and provide cumulative evidence on the reports of EMH in BTM patients. We aim to conduct a systematic review via searching multiple electronic databases (PubMed, Scopus, Google Scholar) to identify eligible articles from any date up to December 2020. Eligible studies should report extramedullary hematopoiesis in BTM. Case reports, case series, observational studies with cross-sectional or prospective research design, case-control studies, and experimental studies will be included if found relevant. Two reviewers (FA and ES) will individually analyze the study quality using the statistical methodology and categories guided by the Cochrane Collaboration Handbook, PRISMA guidelines, and Joanna Briggs Institute checklist for case reports and series. This study will analyze and incorporate the available evidence on EMH in BTM concerning patient demographics, sites of EMH, management, and clinical outcomes of EMH. By summarizing and statistically analyzing the data about EMH in BTM, this study will generate extensive knowledge on the topic for a better understanding of atypical presentations in BTM, a common hemoglobinopathy.
Sections du résumé
BACKGROUND AND AIMS
OBJECTIVE
Thalassemia is one of the most common hemoglobinopathies, with around 5% of the world's population expected to have some degree and type of thalassemia. Beta thalassemia (BT) occurs due to a deficient production of the beta-globin chain of hemoglobin. Extramedullary hematopoiesis (EMH) is one of the complications of BT, mainly observed in minor/intermedia subtypes. EMH is the production of blood cells outside the marrow as a compensatory response to longstanding hypoxia. Due to chronic transfusions, it is not expected in patients with beta-thalassemia major (BTM). However, there are increasingly reported cases of EMH in BTM. The incidence of EMH in BTM is thought to be <1%. However, it seems that the true incidence is much higher than expected. This review aims to pool the available data and provide cumulative evidence on the reports of EMH in BTM patients.
METHODS
METHODS
We aim to conduct a systematic review via searching multiple electronic databases (PubMed, Scopus, Google Scholar) to identify eligible articles from any date up to December 2020. Eligible studies should report extramedullary hematopoiesis in BTM. Case reports, case series, observational studies with cross-sectional or prospective research design, case-control studies, and experimental studies will be included if found relevant. Two reviewers (FA and ES) will individually analyze the study quality using the statistical methodology and categories guided by the Cochrane Collaboration Handbook, PRISMA guidelines, and Joanna Briggs Institute checklist for case reports and series.
RESULTS
RESULTS
This study will analyze and incorporate the available evidence on EMH in BTM concerning patient demographics, sites of EMH, management, and clinical outcomes of EMH.
CONCLUSION
CONCLUSIONS
By summarizing and statistically analyzing the data about EMH in BTM, this study will generate extensive knowledge on the topic for a better understanding of atypical presentations in BTM, a common hemoglobinopathy.
Identifiants
pubmed: 34761125
doi: 10.1002/hsr2.429
pii: HSR2429
pmc: PMC8564650
doi:
Types de publication
Journal Article
Langues
eng
Pagination
e429Informations de copyright
© 2021 The Authors. Health Science Reports published by Wiley Periodicals LLC.
Déclaration de conflit d'intérêts
The authors declare that they have no competing interests.
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