Survival of Thalassemic Patients Referred to the Boo Ali Sina Teaching Hospital, Sari, Iran

Abstract

Beta-thalassemia major (TM) is the most prevalent genetic disease in Mazandaran Province. Currently, about 2,700 TM patients have been registered and are under treatment in the province. This study was undertaken to evaluate the survival of patients attending a dedicated clinic at the Boo Ali Sina Teaching Hospital, Sari, Iran, which was established in 1986. This survival analysis was conducted from July 2004 to September 2006. New deaths were updated in September 2006. A total of 1,010 medical records were reviewed. Place of residence, date of birth, first transfusion, initiation date of chelation, diagnosis of cardiac complications, diabetes and death were recorded. Compliance to treatment assessed by calculating the percentage of actual usage to prescribed iron chelator medicine and also by asking the attending nurse who was constantly present throughout the last 15 years. Validity of the opinion of the nurse was ascertained in a previous study. Kaplan-Meier statistics were used for analysis. The odds ratio (OR) and its 95% confidence interval (CI) for some risk factors of death were calculated. The survival rate of patients born before and after 1986 was also compared. Survival of both genders for birth cohort before and after 1986 was not statistically significant although the cohort of patients born after 1986 was better at 30 years old (68 vs. 80%). The survival of TM patients is improving but the prevalence of complications is high.

Common Complications in Beta-Thalassemia Patients.

“…Similar to our study, Kosaryan et al reported the rate of diastolic dysfunction was 9%; systolic dysfunction, 5%; and severe cardiomyopathy, 5.7%. 21 In a study investigating thalassemia intermedia patients, heart failure was 5.4%; aortic insufficiency, 15.4%; and, mitral valve insufficiency, 47.2%. 22 Cardiac complications were compared with mean ferritin values.…”

The effect of iron chelation therapy on overall survival in sickle cell disease and β‐thalassemia: A systematic review

“…A total of 18 individual papers describing 15 different studies were published between 2000 and 2017, and are summarized in Table . These studies included 7189 patients; however, some patients likely were counted more than once since the multicenter studies from Borgna‐Pignatti et al, although distinct, drew on the same patient populations in Italy .…”

“…Only 1 of the retrospective/longitudinal studies compared survival outcomes in β‐thalassemia patients according to the ICT received; a second study compared monotherapy to combination therapy . The remaining studies investigated survival outcomes according to various baseline and treatment characteristics: 1 investigated factors associated with death in a case‐control study; 4 studied outcomes according to compliance with or duration of ICT; 4 compared survival outcomes according to period of treatment (i.e., before or after availability of different iron chelation treatments); 1 measured survival in patients with thalassemia major and thalassemia intermedia before and after introduction of deferoxamine; and 1 measured rates of death in hepatitis C virus (HCV)‐infected and noninfected patients receiving ICT with deferoxamine …”

“…In the study of patients with or without HCV, nonadherence to chelation treatment was found to be a negative predictive factor of survival (HR, 0.234; 95% CI: 0.075, 0.732; P = .013) . Another retrospective study of patients with β‐thalassemia major in Iran showed that a longer duration of treatment with deferoxamine resulted in significantly longer overall survival . This study is difficult to interpret, however, as little additional information regarding characteristics that predicted survival is provided.…”

“…Furthermore, given the importance of treatment adherence discussed here, whichever treatment an individual patient can administer regularly is likely to be a more important factor to that individual than which agent demonstrates superiority in a head‐to‐head clinical trial. In addition, complications due to iron overload are rare before the second decade of life . However, the average interval between the first appearance of cardiac complications and death in patients with β‐thalassemia ranges from 1 to 2.7 years, depending on the degree of vigilance in screening for heart problems .…”