Splenectomy for people with thalassaemia major or intermedia

Sharma, Akshay; Mathew, Minu; Puri, Latika

doi:10.1002/14651858.cd010517.pub3

Cited by 13 publications

(11 citation statements)

References 41 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Splenectomy is not well proven in beta thalassaemia and raises risks of increased infections; but it can be performed for certain patients who may benefit, with resulting reduction of transfusion requirement. 50 Downsides of the procedure include infections, even years after splenectomy, and thrombotic events postoperatively (both immediate and late). In addition, splenectomised patients may to be at risk for thrombosis on luspatercept, which may be a relative contraindication in the current era.…”

Section: Discussionmentioning

confidence: 99%

Thalassaemia—A global view

Hokland

Daar

Khair³

et al. 2023

Br J Haematol

View full text Add to dashboard Cite

Summary The thalassaemias are a group of genetic disorders of haemoglobin which are endemic in the tropics but are now found worldwide due to migration. Basic standard of care therapy includes regular transfusions to maintain a haemoglobin level of around 10 g/dL, together with iron chelation therapy to prevent iron overload. Novel therapies, bone marrow transplantation, and gene therapy are treatment options that are unavailable in many countries with stressed economies. This Wider Perspectives article presents the strategies for management of an adolescent refugee patient with beta thalassaemia, as it would be performed by expert haematologists in six countries: Italy, Lebanon, Oman, the Sudan, Thailand and the United States. The experienced clinicians in each country have adapted their practice according to the resources available, which vary greatly. Even in the current modern era, providing adequate transfusions and chelation is problematic in many countries. On the other hand, ensuring adherence to therapy, particularly during adolescence, is a similar challenge seen in all countries. The concluding section highlights the disparities in available therapies and puts the role of novel therapies into a societal context.

show abstract

Section: Discussionmentioning

confidence: 99%

Thalassaemia—A global view

Hokland

Daar

Khair³

et al. 2023

Br J Haematol

View full text Add to dashboard Cite

show abstract

“…Patients with thalassemia can undergo splenectomy and cholecystectomy in many phases as part of the therapeutic process. Splenectomy limits the number of blood transfusions and regresses the spread of extramedullary hematopoiesis [26]. Cholelithiasis can occur in thalassemia after Hemoglobin breakdown and the influence of bilirubin deposition in the gallbladder if it becomes symptomatic patients should undergo cholecystectomy with splenectomy [27].…”

Section: Challenges Of Management In Thalassemiamentioning

confidence: 99%

An Overview on Thalassemia Diagnosis and Management Approach, Literature Review

Manea¹,

Khan²,

Alsharyufi³

et al. 2021

Int j pharm res allied sci

View full text Add to dashboard Cite

Thalassemia syndromes are the most common inherited monogenic diseases that kill and affect millions of people worldwide. As Thalassemic diseases became a healthcare system burden for many countries around the world Thalassemia management emerges as a lifetime treatment program that should regularly be monitored with preferable choice for management. In this review we aim to collect and summarize recent articles, studies, and clinical trials in diagnosing and managing thalassemia, and provide a summarized, yet comperhansive study. PubMed database was used for this review and data was collected from relevant journal articles, randomized controlled trials, and observational studies containing the term used in the mesh "Thalassemia" "Management" "Diagnosis" "Laboratory" "Treatment" within the title or abstract. The major pathophysiological change that occurs in thalassemia is the imbalance of the globin chain production. Thalassemia management emerges as a lifetime treatment program that includes intensive medical and surgical care with ongoing monthly clinical visits.

show abstract

“…Present evidence on the best surgical approach between laparotomy and laparoscopy in the setting of HCC are currently limited. However, studies have been published in the particular setting of splenectomy in beta-thalassemia patients [ 81 , 82 ]. A single randomized study comparing laparotomic vs. laparoscopic splenectomy showed an increased incidence of intraoperative and postoperative bleedings in the laparoscopic group [ 83 ].…”

Section: Main Bodymentioning

confidence: 99%

Hepatocellular carcinoma in adult thalassemia patients: an expert opinion based on current evidence

et al. 2020

View full text Add to dashboard Cite

Beta-thalassemia represents a heterogeneous group of haemoglobin inherited disorders, among the most common genetic diseases in the world, frequent in the Mediterranean basin. As beta-thalassemia patients' survival has increased over time, previously unknown complications are observed with increasing frequency. Among them, an increased risk of hepatocellular carcinoma (HCC) has been registered. Our aim is to reduce inequalities in diagnosis and treatment and to offer patients univocal recommendations in any institution. The members of the panel-gastroenterologists, radiologists, surgeons and oncologists-were selected on the basis of their publication records and expertise. Thirteen clinical questions, derived from clinical needs, and an integration of all the committee members' suggestions, were formulated. Modified Delphi approach involving a detailed literature review and the collective judgement of experts, was applied to this work. Thirteen statements were derived from expert opinions' based on the current literature, on recently developed reviews and on technological advancements. Each statement is discussed in a short paragraph reporting the current key evidence. As this is an emerging issue, the number of papers on HCC in beta-thalassemia patients is limited and based on anecdotal cases rather than on randomized controlled studies. Therefore, the panel has discussed, step by step, the possible differences between beta-thalassemia and non beta-thalassemia patients. Despite the paucity of the literature, practical and concise statements were generated. This paper offers a practical guide organized by statements describing how to manage HCC in patients with betathalassemia.

show abstract

Splenectomy for people with thalassaemia major or intermedia

Cited by 13 publications

References 41 publications

Thalassaemia—A global view

Thalassaemia—A global view

An Overview on Thalassemia Diagnosis and Management Approach, Literature Review

Hepatocellular carcinoma in adult thalassemia patients: an expert opinion based on current evidence

Contact Info

Product

Resources

About