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. 2020 Apr 14;4(7):1448-1457.
doi: 10.1182/bloodadvances.2019000760.

Life expectancy and risk factors for early death in patients with severe thalassemia syndromes in South India

Rakesh Dhanya  1 , Amit Sedai  1   2 , Kumari Ankita  1 , Lalith Parmar  1   2 , Rajat Kumar Agarwal  1   2 , Santhosh Hegde  1 , Gayathri Ramaswami  3 , Ashwini Gowda  3 , S Girija  3 , Pooja Gujjal  4 , H Pushpa  1   4 , J Dasaratha Ramaiah  5 , Chandrakala Karri  5 , Sujata Jali  6 , Neelavva Rayappa Tallur  6 , U V Shenoy  7 , Diana Pinto  7 , Stalin Ramprakash  8 , C P Raghuram  8 , Deepa Trivedi  9 , Xueyuan Cao  10 , Lawrence Faulkner  1   11
Affiliations

Life expectancy and risk factors for early death in patients with severe thalassemia syndromes in South India

Rakesh Dhanya et al. Blood Adv. .

Abstract

In spite of advances in chelation therapy and screening of blood, mortality associated with the most common life-threatening noncommunicable disease of children in India, transfusion-dependent thalassemia (TDT), remains poorly defined. This study aims at estimating death rates and mortality risk factors associated with TDT. The clinical records of 1087 patients from 5 thalassemia centers in India were retrospectively analyzed from 2011 to 2018. Median patient age was 8.5 years, with 107 patients older than 18 years; 656 patients were male and 431 were female. Demographic details and clinical parameters were analyzed at presentation and at last visit. With 41 recorded deaths, actuarial survival at 26.9 years was 50%, and under-5 mortality was 7 times higher than in the general population. Patients with transfusion-transmitted infections (TTIs) had 3.4 times higher risk for death (P = .031). Serum ferritin higher than 4000 ng/dL had 4.6 times higher risk for mortality compared with ferritin lower than 1000 ng/dL (P = .00063). A hemoglobin drop lower than 2 g/dL per week had 7.7 times higher mortality risk compared with a drop of less than 1 g/dL per week (P < .0001). Social determinants (sex, economic status, and distance from center), splenectomy, and even cardiac complications were not associated with higher mortality risk. Main causes of death were infection, iron overload, TTIs, and allo-immunization. Patients who received more than 4 years of adequate care had more than 66% mortality risk reduction (P < .0001). TDT in India continues to result in high mortality. Ineffective transfusion, TTIs, and chelation continue to be the most significant risk factors. Comprehensive care in dedicated day care centers from early age is likely to improve outcomes.

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Conflict of interest statement

Conflict-of-interest disclosure: The authors declare no competing financial interests.

Figures

None
Graphical abstract
Figure 1.
Figure 1.
The overall survival of 1087 transfusion-dependant patients with thalassemia across 5 centers.
Figure 2.
Figure 2.
Categorical comparison of survival. (A) Surival of patients born before and after 2000. (B) Survival between sexes. (C) Survival according to economic status. (D) Survival based on distance from transfusion center. (E) Survival between patients based on age of start of transfusion. (F) Survival according to TTI status. (G) Survival based on splenectomy status. (H) Survival according to hepatitis B vaccination status. (I) Survival based on cardiac status. (J) Survival against different classes of serum iron levels. (K) Survival difference according to rate of fall of Hb per week. (L) Survival based on length of follow up at day care.
Figure 2.
Figure 2.
Categorical comparison of survival. (A) Surival of patients born before and after 2000. (B) Survival between sexes. (C) Survival according to economic status. (D) Survival based on distance from transfusion center. (E) Survival between patients based on age of start of transfusion. (F) Survival according to TTI status. (G) Survival based on splenectomy status. (H) Survival according to hepatitis B vaccination status. (I) Survival based on cardiac status. (J) Survival against different classes of serum iron levels. (K) Survival difference according to rate of fall of Hb per week. (L) Survival based on length of follow up at day care.
Figure 3.
Figure 3.
Causes of death categorized by age.
See this image and copyright information in PMC

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