Clinical and Haematological Picture of Multi-Transfused Thalassaemia Major Patients at a Center in Pakistan

Objective: To describe the clinical and haematological picture of multiply transfused patients of thalassaemia major at a treatment center in Pakistan. Study Design: Descriptive cross-sectional. Place and Duration of Study: The study was conducted at Thalassaemia Treatment Centre Rawalpindi. The stu...

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Main Author: Suhaib Ahmed , Zohra Jabeen Wazir , Ishrat Abdul Qayyum
Format: Article
Language:English
Published: Riphah International University, Islamabad 2018-06-01
Series:Journal of Islamic International Medical College
Subjects:
Online Access:https://journals.riphah.edu.pk/index.php/jiimc/article/view/1029
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author Suhaib Ahmed , Zohra Jabeen Wazir , Ishrat Abdul Qayyum
author_facet Suhaib Ahmed , Zohra Jabeen Wazir , Ishrat Abdul Qayyum
author_sort Suhaib Ahmed , Zohra Jabeen Wazir , Ishrat Abdul Qayyum
collection DOAJ
description Objective: To describe the clinical and haematological picture of multiply transfused patients of thalassaemia major at a treatment center in Pakistan. Study Design: Descriptive cross-sectional. Place and Duration of Study: The study was conducted at Thalassaemia Treatment Centre Rawalpindi. The study was done between Jan 1994 and May 2017. Materials and Methods: A total of 383 patients of thalassaemia major (TM) on regular blood transfusions were examined for various clinical and haematological parameters. In addition case records of 101 patients of TM who died during treatment were also studied. Results: In the 383 patients on treatment 328 (86%) were born to consanguineous parents, 246 (64%) were from lower socio-economic group, 85 (22%) had one or more affected siblings, 145 (38%) had hepatomegaly, 191 (50%) had splenomegaly, 42 (11%) had undergone splenectomy and 187 (49%) had never received iron chelation therapy. Height and weight in the 383 patients on treatment showed marked stunting. Median age of the 383 patients on treatment was 104 months as compared to 119 months in the deceased group of patients (p=0.0263). Pre-transfusion Hb in the alive patients (7.1 g/dL) was higher than in the deceased patients (6.4 g/dL) (p=0.0142). Ferritin level in the patients on treatment (3698 μg/L) was lower than in the deceasedpatients (4616 μg/L) (p=0.0069). In the 383 patients on treatment 141 (36.8%) were HCV positive. Conclusion: Majority of the patients at a thalassaemia treatment center are chronically under-transfused and have moderate to severe growth retardation. They get inadequate iron chelation resulting in high mortality before tenth year of life.
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spelling doaj.art-ef227aee0406425f93bc421fc4be662b2022-12-22T03:33:09ZengRiphah International University, IslamabadJournal of Islamic International Medical College1815-40182410-54222018-06-0113252561425Clinical and Haematological Picture of Multi-Transfused Thalassaemia Major Patients at a Center in PakistanSuhaib Ahmed , Zohra Jabeen Wazir , Ishrat Abdul QayyumObjective: To describe the clinical and haematological picture of multiply transfused patients of thalassaemia major at a treatment center in Pakistan. Study Design: Descriptive cross-sectional. Place and Duration of Study: The study was conducted at Thalassaemia Treatment Centre Rawalpindi. The study was done between Jan 1994 and May 2017. Materials and Methods: A total of 383 patients of thalassaemia major (TM) on regular blood transfusions were examined for various clinical and haematological parameters. In addition case records of 101 patients of TM who died during treatment were also studied. Results: In the 383 patients on treatment 328 (86%) were born to consanguineous parents, 246 (64%) were from lower socio-economic group, 85 (22%) had one or more affected siblings, 145 (38%) had hepatomegaly, 191 (50%) had splenomegaly, 42 (11%) had undergone splenectomy and 187 (49%) had never received iron chelation therapy. Height and weight in the 383 patients on treatment showed marked stunting. Median age of the 383 patients on treatment was 104 months as compared to 119 months in the deceased group of patients (p=0.0263). Pre-transfusion Hb in the alive patients (7.1 g/dL) was higher than in the deceased patients (6.4 g/dL) (p=0.0142). Ferritin level in the patients on treatment (3698 μg/L) was lower than in the deceasedpatients (4616 μg/L) (p=0.0069). In the 383 patients on treatment 141 (36.8%) were HCV positive. Conclusion: Majority of the patients at a thalassaemia treatment center are chronically under-transfused and have moderate to severe growth retardation. They get inadequate iron chelation resulting in high mortality before tenth year of life.https://journals.riphah.edu.pk/index.php/jiimc/article/view/1029blood transfusion, complications, pakistan, survival, thalassemia.
spellingShingle Suhaib Ahmed , Zohra Jabeen Wazir , Ishrat Abdul Qayyum
Clinical and Haematological Picture of Multi-Transfused Thalassaemia Major Patients at a Center in Pakistan
Journal of Islamic International Medical College
blood transfusion, complications, pakistan, survival, thalassemia.
title Clinical and Haematological Picture of Multi-Transfused Thalassaemia Major Patients at a Center in Pakistan
title_full Clinical and Haematological Picture of Multi-Transfused Thalassaemia Major Patients at a Center in Pakistan
title_fullStr Clinical and Haematological Picture of Multi-Transfused Thalassaemia Major Patients at a Center in Pakistan
title_full_unstemmed Clinical and Haematological Picture of Multi-Transfused Thalassaemia Major Patients at a Center in Pakistan
title_short Clinical and Haematological Picture of Multi-Transfused Thalassaemia Major Patients at a Center in Pakistan
title_sort clinical and haematological picture of multi transfused thalassaemia major patients at a center in pakistan
topic blood transfusion, complications, pakistan, survival, thalassemia.
url https://journals.riphah.edu.pk/index.php/jiimc/article/view/1029
work_keys_str_mv AT suhaibahmedzohrajabeenwazirishratabdulqayyum clinicalandhaematologicalpictureofmultitransfusedthalassaemiamajorpatientsatacenterinpakistan