Managing thalassemia in the developing world: an evidence-based approach for prevention, transfusion independency, and curative treatment with hematopoietic stem cell transplantation

Ansari, Saqib Hussain; Parveen, Sadia; Siddiqui, Saima; Perveen, Kousar; Ahmed, Ghazala; Kaleem, Bushra; Ahmed, Shariq; Zohaib, Muhammad; Farzana, Tasneem; Shamsi, Tahir

doi:10.1182/bloodadvances.2018gs112057

Cited by 11 publications

(10 citation statements)

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“…Red cell phenotyping and pre-storage leucodepletion are not done by all the thalassemia centers in Pakistan, resulting in alloimmunization and increases transfusion requirements, which further increases the risk of TTIs [40]. A high cost of standardized care of TM ($4500 per year) is an additional factor contributing to a higher rate of TTIs in Pakistan where the average annual income of individual families is less than $587 with more than 35% of the population living below the poverty line [18,41]. The majority of TM patients cannot afford the cost of regular BTs and have limited access to thalassemia centers [30].…”

Section: Discussionmentioning

confidence: 99%

“…The rate of infections in TM patients may serve as a marker for the high prevalence of TTIs in the general population. Several studies have reported a variable rate of TTIs among TM patients in Pakistan, and the exact prevalence is still undetermined [16][17][18][19]. The purpose of this systematic review is to analyze the available data and evaluate the overall prevalence of TTIs in TM patients chronically dependent on blood transfusions.…”

Section: Introductionmentioning

confidence: 99%

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Prevalence of Transfusion Transmissible Infections in Beta-Thalassemia Major Patients in Pakistan: A Systemic Review

Ehsan¹,

Wahab²,

Anwer³

et al. 2020

Cureus

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β-thalassemia major (TM) is one of the most prevalent inherited hemoglobinopathies in Pakistan. It has one of the highest prevalence of transfusion-dependent TM patients globally, with an estimated greater than 100,000 active cases. Blood transfusions (BT) are essential in the management of severe TM; it is critical to have a safe BT to reduce the risk of transfusion transmissible infections (TTIs). Frequent blood transfusions in these patients increase their risk of acquiring TTIs compared to the general population. We performed a systematic literature search to identify studies related to the TTIs and transfusion-related infections in Pakistan from January 1, 2010, to January 31, 2020. The search was conducted using PubMed and PakMediNet, with initial search retrieved 981 studies. Among these, 166 studies met the inclusion criteria, and only 14 studies met the final criteria for qualitative synthesis. Analysis of 14 studies (n = 3786) showed the seroprevalence of hepatitis B virus (HBV) of 3.13% (0.66% to 7.4%) and hepatitis C virus (HCV) of 26% (5.56% to 68.2%). There were only two studies that reported HIV seroprevalence of 0% and 0.5% (n = 6). The rate of seropositivity for HBV and HCV was directly related to the number of transfusions, higher ferritin levels, and older age groups. There was an increase in the HCV rate with the increasing age of patients. Thalassemia patients, who were older than ten years of age, had an HCV rate of 22% compared to only 8.4% in patients younger than ten years of age. A comparison of HCV in healthy donors vs. thalassemia patients showed a rate of 1.9% vs. 13.1% for TM patients. The majority of the patients were males (51% to 88%). The seroprevalence of TTIs was higher in males than in females (73.4% vs. 26.6%). On average, a single TM patient is exposed to at least 17 different donors annually, requiring 1-2 transfusions every month. Our study highlights that the prevalence of transfusion-transmitted infections, especially HCV, is alarmingly higher (26%) in the TM population than in the general population. There is limited data regarding the prevalence of HIV, syphilis, and malaria in this population. This is mainly due to a fragmented system of blood transfusion, weak regulations, and lower rates of voluntary blood donations. These findings warrant better health measures to improve the blood donation system and specialized care for TM patients.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Prevalence of Transfusion Transmissible Infections in Beta-Thalassemia Major Patients in Pakistan: A Systemic Review

Ehsan¹,

Wahab²,

Anwer³

et al. 2020

Cureus

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“…17 Preventing the birth of newborns with thalassemia major through prenatal diagnosis is widely accepted as the most approach for the prevention of thalassemia. 18 Since the National Free Thalassemia Control Programme was initiated on December 2013, premarital, pregestational, and prenatal screening of thalassemia together with intensification of health education of thalassemia-related knowledge has been conducted in highly prevalent areas across Fujian province, aiming to reduce the prevalence of thalassemia in newborns. 19 During the 10-year study period from 2009 through 2018, the number of fetus undergoing prenatal diagnosis was approxi- ulations may increase the detection of thalassemia, which is effective to reduce the incidence of birth defects.…”

Section: Discussionmentioning

confidence: 99%

Prenatal diagnosis of thalassemia in 695 pedigrees from southeastern China: a 10‐year follow‐up study

Huang

Chen

et al. 2021

Clinical Laboratory Analysis

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“…A particular classfication of thalassemia, beta-thalassemia major, is described as the most common genetic disorder in Pakistan. It is estimated to have nine million carriers, while 40,000 children are registered as transfusion dependent and 5,000-9,000 children are born annually with the condition (Ahmed et al 2021;Ansari et al 2018). During the last decade, prenatal diagnostic services for thalassemia alongside other congenital conditions have been on the rise within both the public and private sector in a number of major cities in Pakistan.…”

Section: Introductionmentioning

confidence: 99%

More than sample providers: how genetic researchers in Pakistan mobilized a prenatal diagnostic service for thalassemia

Sheikh

Wahlberg

2021

BioSocieties

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While unequally resourced partners from the so-called global South are often considered ‘mere sample providers’ in larger international genomics collaborations, in this paper, we show how they strategically work to mobilize their role in a global system of tissue exchange to deliver services for local communities. We unpack how a prenatal diagnostic service for thalassemia in Pakistan emerged out of the maneuvering efforts of internationally connected Pakistani researchers. By tracing the distributed capacities that emerged and circulated as they set about improving medical genetics in Pakistan, we outline some key conditions that led to the establishment of the service: first, the scale of unmet needs that geneticists faced when collecting data as part of their research that made medical genomics a relevant field; secondly, joint efforts between researchers and physicians that were engaged with the challenge of decreasing disease prevalence through diagnostics and abortion; and finally, the ways in which international research collaborations helped generate resources to improve medical genetics in Pakistan. To understand how genetic research and medicine is currently being developed in Pakistan, we need to ethnographically re-center our analyses in ways that allow us to identify the resourceful ways in which researchers maneuvre to secure locally relevant outcomes.

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Managing thalassemia in the developing world: an evidence-based approach for prevention, transfusion independency, and curative treatment with hematopoietic stem cell transplantation

Cited by 11 publications

References 5 publications

Prevalence of Transfusion Transmissible Infections in Beta-Thalassemia Major Patients in Pakistan: A Systemic Review

Prevalence of Transfusion Transmissible Infections in Beta-Thalassemia Major Patients in Pakistan: A Systemic Review

Prenatal diagnosis of thalassemia in 695 pedigrees from southeastern China: a 10‐year follow‐up study

More than sample providers: how genetic researchers in Pakistan mobilized a prenatal diagnostic service for thalassemia

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