Clinical and Haematological Picture of Multi-Transfused Thalassaemia Major Patients at a Center in PakistanSuhaib Ahmed1, Zohra Jabeen Wazir2, Ishrat Abdul Qayyum.
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 deceased
patients (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
Key words: Blood Transfusion, Complications, Pakistan, Survival, Thalassemia