Transfusion-naïve thalassemia minor/trait is often associated with decreased risk of coronary artery diseases. The present study aimed to evaluate the effect of transfusion-naïve thalassemia on the incidence of arterial thromboembolic events using the National Health Insurance Research Database, Taiwan (2001–2010). Data from patients with transfusion-naïve thalassemia (n=2356) frequency matched with non-thalassemia subjects (n=9424) according to sex, age, and index year at a ratio of 1:4 were included. The risk of arterial thromboembolic events, cerebrovascular ischemic events, arterial embolism/thrombosis, peripheral embolism, myocardial infarction, myocardial ischemia, and angina pectoris in transfusion-naïve thalassemia were analyzed using Cox proportional hazard regression models. The transfusion-naïve thalassemia group had significantly higher risk of arterial thromboembolic events (aHR=1.28, 95% CI 1.07 to 1.52) and myocardial ischemia (aHR=1.41, 95% CI 1.13 to 1.76) as compared with the non-thalassemia group. In addition, they also had a significantly higher cumulative incidence of arterial thromboembolic event and myocardial ischemia. Interestingly, a higher risk of arterial thromboembolic events (aHR=1.58, 95% CI 1.22 to 2.04) and myocardial ischemia (aHR=1.73, 95% CI 1.25 to 2.41) was observed in men with thalassemia as compared with those without. Furthermore, patients with comorbidities had an increased risk of arterial thromboembolic events than did those without comorbidities. The effect of thalassemia on arterial thromboembolic events may be mainly attributed to the influence of thalassemia on myocardial ischemia, as no significant differences were observed in other outcomes evaluated in the present study. In conclusion, the present study confirms the increased risk of arterial thromboembolic events, mainly attributed to the dramatic increase in myocardial ischemia, inminor patients with transfusion-naïve thalassemia.
Background : Fludarabine and busulfan as a reduced-toxicity Conditioning regimen has been widely used for hematopoietic stem cell transplantation(HSCT) in pediatric β-thalassemia patients,however,the data of transplant-related infections in this population are scare. Methods:This retrospective study assessed the clinical features of infections in 265 children who received HSCT in pediatric department of Nanfang Hospital from January 2011 to December 2015,including 151 with allogeneic hematopoietic stem cell transplantation(allo-HSCT) from unrelated donors(UDs) who are all well-matched human leukocyte antigens, 87 with HSCT from matched sibling donors(MSDs),27 with genetic haploid transplantations from matched donors(father donors=13,mother donors=13,aunt donor=1). the median age was 6 years(range:1.7 to 16 years),and the ratio of male to female patients was 1.6(163:102).The median follow-up time was 35 months(range:8-68 months). Results: The cumulative incidence of neutrophil recovery was 97.70%,98.01% and 100% in MSD-HSCT,UD-PBSCT and genetic haploid,respectively.Most of the infections took place in the oral cavity(58.72%),respiratory tract, digestive tract and skin.Bacteremia developed in 46(17.36%) patients and 67.39% happened within 30 day of transplantation,the median time was +3 day(rang-2 to +11) when Bone marrow is barren period. G_ bacteria(68%) were the most commom causes of infections;Escherichia coli,Klebsiella pneumoniae and Pseudomonas aeruginosa accounted for the most of G- bacteria infections.Fungal turn out in 16(6.04%) patients,25% of the people was diagnosed with a probable,invasive fungal disease.The cumulative incidence of cytomegalovirus(CMV) and herpes zoster was 4.9% and17.36% .The cumulative incidence of grade II-IV aGVHD was 4.26%.17 patients died,9 of them suffered GVHD and complicated with severe pneumonia, 3 due to infection complicated with heart failure,4 of them were attributed to severe infections lead to multiple organ failure,and one appeared pancytopenia 6 months after transplantation , combined EB virus infection, cytomegalovirus infection, However, the use of MabThera treatment ineffective ,he died of liver failure,and brain hemorrhage.The estimate 5-year overall survival were 95%,94% and 84% in MSD-HSCT,UD-PBSCT and genetic haploid,respectively. Conclusions:In conclussion,our data suggests that the myeloablative conditioning regimen transplantation based on fludarabine and busulfan provided an effective measure for pediatric β-thalassemia patients with infections comparable to other transplant center reports but higher overall survival and disease free survival. More efforts should be made to rational use of antimicrobial drugs, empirical antifungal agents and strengthen the mouth, lungs, anus and other vulnerable parts of the care ,especially after the transplant neutropenic period. Disclosures No relevant conflicts of interest to declare.
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