Dutch-beta thalassemia: A rare mutation from India
Introduction: COVID-19 pandemic imposed challenges towards management of transfusion-dependent thalassemia patients (TDT). The need for regular blood transfusions and iron chelation therapy in these patients added further uncertainty about managing COVID-19 in this subset of patients. Aims: To descr...
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| Format: | Article |
| Language: | English |
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Elsevier
2022-09-01
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| Series: | Pediatric Hematology Oncology Journal |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2468124522002339 |
| _version_ | 1798003223372496896 |
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| author | Nirali Sanghvi Priyanka Aggarwal Vineeta Singh Vineeta Gupta |
| author_facet | Nirali Sanghvi Priyanka Aggarwal Vineeta Singh Vineeta Gupta |
| author_sort | Nirali Sanghvi |
| collection | DOAJ |
| description | Introduction: COVID-19 pandemic imposed challenges towards management of transfusion-dependent thalassemia patients (TDT). The need for regular blood transfusions and iron chelation therapy in these patients added further uncertainty about managing COVID-19 in this subset of patients. Aims: To describe the clinical manifestations of SARS-CoV2 infection in patients with TDT and to evaluate feasibility of home management for patients with mild disease. Materials and methods: The study involved TDT patients registered with thalassemia day care center, DMCH, who tested positive for COVID-19 by RTPCR. The demographics, clinical characteristics and baseline investigations were recorded. Patients with mild disease were managed at home and others were hospitalized. The daily home monitoring and the hospital course were noted and analyzed. Results: The study involved 14 TDT patients who were infected with SARS-CoV2 with a mean age of 18.9 ± 6.7 years and a male to female ratio of 6:1. Five patients each were in low and high-risk groups and 4 patients were in highest risk group. The symptoms reported by these patients were fever, fatigue, sore throat etc. Two patients were hospitalized with one patient requiring oxygen therapy. He was discharged after 48 hours. The other patient had severe cardiac iron overload and diabetes mellitus. His iron chelation therapy was with held during hospitalization. He presented with a cardiac arrhythmia later and was cardioverted. Thus, all other patients were continued on iron chelation with deferasirox. Twelve patients were successfully managed at home with regular telephonic monitoring. Conclusion: Patients with thalassemia do not necessarily need hospitalization for management of COVID-19. Home management can be offered to patients with mild disease in a resource limited setting. Iron chelation with deferasirox can be continued safely. |
| first_indexed | 2024-04-11T12:04:27Z |
| format | Article |
| id | doaj.art-d133cbbc70884ed7a5f289afc1837989 |
| institution | Directory Open Access Journal |
| issn | 2468-1245 |
| language | English |
| last_indexed | 2024-04-11T12:04:27Z |
| publishDate | 2022-09-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Pediatric Hematology Oncology Journal |
| spelling | doaj.art-d133cbbc70884ed7a5f289afc18379892022-12-22T04:24:45ZengElsevierPediatric Hematology Oncology Journal2468-12452022-09-01737476Dutch-beta thalassemia: A rare mutation from IndiaNirali Sanghvi0Priyanka Aggarwal1Vineeta Singh2Vineeta Gupta3Corresponding author. Division of Pediatric Hematology-Oncology, Department of Pediatrics, Institute of Medical Sciences, Banaras Hindu University, Lanka, Varanasi, 221005, India.; Division of Pediatric Hematology-Oncology/BMT, Department of Pediatrics, Institute of Medical Sciences, Banaras Hindu University, Varanasi, 221005, Uttar Pradesh, IndiaDivision of Pediatric Hematology-Oncology/BMT, Department of Pediatrics, Institute of Medical Sciences, Banaras Hindu University, Varanasi, 221005, Uttar Pradesh, IndiaDivision of Pediatric Hematology-Oncology/BMT, Department of Pediatrics, Institute of Medical Sciences, Banaras Hindu University, Varanasi, 221005, Uttar Pradesh, IndiaDivision of Pediatric Hematology-Oncology/BMT, Department of Pediatrics, Institute of Medical Sciences, Banaras Hindu University, Varanasi, 221005, Uttar Pradesh, IndiaIntroduction: COVID-19 pandemic imposed challenges towards management of transfusion-dependent thalassemia patients (TDT). The need for regular blood transfusions and iron chelation therapy in these patients added further uncertainty about managing COVID-19 in this subset of patients. Aims: To describe the clinical manifestations of SARS-CoV2 infection in patients with TDT and to evaluate feasibility of home management for patients with mild disease. Materials and methods: The study involved TDT patients registered with thalassemia day care center, DMCH, who tested positive for COVID-19 by RTPCR. The demographics, clinical characteristics and baseline investigations were recorded. Patients with mild disease were managed at home and others were hospitalized. The daily home monitoring and the hospital course were noted and analyzed. Results: The study involved 14 TDT patients who were infected with SARS-CoV2 with a mean age of 18.9 ± 6.7 years and a male to female ratio of 6:1. Five patients each were in low and high-risk groups and 4 patients were in highest risk group. The symptoms reported by these patients were fever, fatigue, sore throat etc. Two patients were hospitalized with one patient requiring oxygen therapy. He was discharged after 48 hours. The other patient had severe cardiac iron overload and diabetes mellitus. His iron chelation therapy was with held during hospitalization. He presented with a cardiac arrhythmia later and was cardioverted. Thus, all other patients were continued on iron chelation with deferasirox. Twelve patients were successfully managed at home with regular telephonic monitoring. Conclusion: Patients with thalassemia do not necessarily need hospitalization for management of COVID-19. Home management can be offered to patients with mild disease in a resource limited setting. Iron chelation with deferasirox can be continued safely.http://www.sciencedirect.com/science/article/pii/S2468124522002339COVID-19Transfusion dependent thalassemiaIron chelation therapyDeferasirox |
| spellingShingle | Nirali Sanghvi Priyanka Aggarwal Vineeta Singh Vineeta Gupta Dutch-beta thalassemia: A rare mutation from India Pediatric Hematology Oncology Journal COVID-19 Transfusion dependent thalassemia Iron chelation therapy Deferasirox |
| title | Dutch-beta thalassemia: A rare mutation from India |
| title_full | Dutch-beta thalassemia: A rare mutation from India |
| title_fullStr | Dutch-beta thalassemia: A rare mutation from India |
| title_full_unstemmed | Dutch-beta thalassemia: A rare mutation from India |
| title_short | Dutch-beta thalassemia: A rare mutation from India |
| title_sort | dutch beta thalassemia a rare mutation from india |
| topic | COVID-19 Transfusion dependent thalassemia Iron chelation therapy Deferasirox |
| url | http://www.sciencedirect.com/science/article/pii/S2468124522002339 |
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