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

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.

Tuberculosis Coexistence in Pediatric Hodgkin's Lymphoma: A Tropical Country Experience.

Introduction Hodgkin's lymphoma (HL), being one of the common cancers among children, may occasionally masquerade as an infectious illness. Similarly, an underlying infection like tuberculosis (TB) may be missed in cases of HL because of similarity in clinical and radiological features. Here, we present our data of association of HL with histopathologically proven TB lymph node, their clinical presentation, treatment details, and outcome. Materials and Methods A retrospective review of all the cases of HL diagnosed between January 2007 and December 2016 was done. The cases which had an association of TB, based on the histopathology, were reviewed separately. Results A total of 262 children with HL were treated at our institute from January 2007 to December 2016. Of these cases, 42 children had received empirical antitubercular therapy (ATT) (due to suspicion of TB) before presenting to us, and only five cases had histopathologically proven TB lymph node. Ziehl-Neelsen (ZN) stain for acid-fast bacilli (AFB) was positive in the biopsy specimen of all the five cases, proving TB lymph node coexistence with HL. They were treated with six-drug ATT as per the Revised National Tuberculosis Control Program (RNTCP) guidelines along with chemotherapy with adriamycin, bleomycin, vinblastine, and dacarbazine regimen. All the five patients are healthy and disease free until their last follow-up. Conclusion A high-end suspicion for concomitant TB and HL is needed, especially in our country where TB is still rampant. Biopsy with immunohistochemistry and demonstration of AFB can enable a definite diagnosis of both the entities.

Information Technology-Assisted Treatment Planning and Performance Assessment for Severe Thalassemia Care in Low- and Middle-Income Countries: Observational Study.

BACKGROUND: Successful models of information and communication technology (ICT) applied to cost-effective delivery of quality care in low- and middle-income countries (LMIC) are an increasing necessity. Severe thalassemia is one of the most common life-threatening noncommunicable diseases of children globally. OBJECTIVE: The aim was to study the impact of ICT on quality of care for severe thalassemia patients in LMIC. METHODS: A total of 1110 patients with severe thalassemia from five centers in India were followed over a 1-year period. The impact of consistent use of a Web-based platform designed to assist comprehensive management of severe thalassemia (ThalCare) on key indicators of quality of care such as minimum (pretransfusion) hemoglobin, serum ferritin, liver size, and spleen size were assessed. RESULTS: Overall improvements in initial hemoglobin, ferritin, and liver and spleen size were significant (P<.001 for each). For four centers, the improvement in mean pretransfusion hemoglobin level was statistically significant (P<.001). Four of five centers achieved reduction in mean ferritin levels, with two displaying a significant drop in ferritin (P=.004 and P<.001). One of the five centers did not record liver and spleen size on palpation, but of the remaining four centers, two witnessed a large drop in liver and spleen size (P<.01), one witnessed moderate drop (P=.05 for liver; P=.03 for spleen size), while the fourth witnessed a moderate increase in liver size (P=.08) and insignificant change in spleen size (P=.12). CONCLUSIONS: Implementation of computer-assisted treatment planning and performance assessment consistently and positively impacted indexes reflecting effective delivery of care to patients suffering from severe thalassemia in LMIC.