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

TitleLife expectancy and risk factors for early death in patients with severe thalassemia syndromes in South India.
Publication TypeJournal Article
Year of Publication2020
AuthorsDhanya, R, Sedai, A, Ankita, K, Parmar, L, Agarwal, RK, Hegde, S, Ramaswami, G, Gowda, A, Girija, S, Gujjal, P, Pushpa, H, J Ramaiah, D, Karri, C, Jali, S, Tallur, NRayappa, Shenoy, UV, Pinto, D, Ramprakash, S, Raghuram, CP, Trivedi, D, Cao, X, Faulkner, L
Publication NameBlood Adv
Volume4
Issue7
Pagination1448-1457
Date Published2020 Apr 14
ISSN2473-9537
KeywordsC2C bibliography, India, Prognostic factors, supportive care, Thalassemia
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

DOI10.1182/bloodadvances.2019000760
Alternate JournalBlood Adv
PubMed ID32282881
PubMed Central IDPMC7160270