The survival rate of patients with beta-thalassemia major and intermedia and its trends in recent years in Iran

Abstract

OBJECTIVESThalassemia is a common genetic disease in Iran, especially in the north and south of Iran. The present study sought to determine the survival rate of patients with thalassemia in highly endemic regions of Iran and its variation in patients born before and after 1971.METHODSThe present historical cohort study extracted data from the health records of patients with beta-thalassemia major, beta-thalassemia intermedia, and sickle beta-thalassemia who had presented to thalassemia treatment centers in the past years. The collected data were analyzed using the Kaplan-Meier test, the log-rank test, and the chi-square test.RESULTSOf the total of 5,491 medical records (2,647 men and 2,634 women; mean age, 23.81±11.32 years), 3,936 belonged to patients with beta-thalassemia major, and 999 and 89 to patients with beta-thalassemia intermedia and sickle beta-thalassemia, respectively. In 467 cases, the type of thalassemia was not clear. The cumulative survival rate was calculated as 0.92, 0.83, 0.74, and 0.51 by ages 25, 35, 45, and 55, respectively. The hazard ratio of death was 4.22 (p<0.05) for beta-thalassemia major and 0.77 for beta-thalassemia intermedia (p=0.70). It was calculated as 1.45 for men patients and as 3.82 for single patients.CONCLUSIONSThe present study showed relatively high survival rates in patients with thalassemia. The survival of patients was unfavorable in poorer regions (Zahedan and Iranshahr). Factors including women gender, a higher level of education, being married, and living in metropolises decreased the risk of death at younger ages and improved survival.

Global survival rate of patients with beta thalassemia major: a systematic review and meta-analysis

“…The included studies were published from 1994 to 2018. Base on geographical location, five studies conducted in Iran 9,[20][21][22][23] , one in Italy, 24 and one in Cyprus. 25 Characteristics of the included studies presented in Table 2.…”

“…9 Some factors affect on this increasing picture of TM survival including improving of surveillance in some parts of the world, improved treatment of cardiac complications, better accessibility to blood donor, blood screening for pathogens, treatment of infections, and treatment with deferoxamine. 10,11 In the Modell et al study, decreasing of cardiac disease in TM was reported as the main factor in increasing of survival. 12 Lower survival rate in some reigns in the world associated with availability of deferoxamine treatment and also related to burdensome iron chelation regimen refusal by TM patients.…”

Thalassemia-Beta major-Case report

“…Signifi cant success has been achieved with allogeneic bone marrow transplantation in patients with homozygous -thalassemia who have had an HLA-identical donor. The mortality from the procedure in these patients is quite high (30%), due to the complications of iron overload, but it is signifi cantly lower (below 10%) if the intervention is performed in early childhood [8]. The orofacial manifestations in people with thalassemia are numerous and intense.…”

Prevalence and risk factors of fractures in transfusion dependent thalassemia – A Hong Kong Chinese population cohort

“…Prior report showed 381 patients required regular transfusion in Hong Kong 2 . Survival in patients with transfusion dependent thalassemia (TDT) improved with optimized care and newer iron chelating agents, long‐term complications surfaced as a result 3 . Bone health has become a major issue in the past decades as this specific cohort is aging 4 .…”