Study on the Clinico-Haematological Profile of Patients of Non-Transfusion Dependent Thalassemia (NTDT) Attending Thalassemia Clinic at a Tertiary Care Hospital.

Corresponding Author:
Indrani Das,
Flat No. 0/4, Cluster 8,
Purbachal Housing, Salt Lake,
Sector 3, Kolkata-700097,
West Bengal, India.
E-mail: dr_indranidas@rediffmail.com

ABSTRACT

BACKGROUND

Non-Transfusion Dependent Thalassemia (NTDT) is a term used to label patients who do not require lifelong transfusions for survival. The common conditions traditionally described as NTDT are beta thalassemia intermedia, haemoglobin H disease and haemoglobin E beta thalassemia. The major problem with NTDT patients remains that, as they do not require regular transfusions, so they often do not seek medical attention until they develop serious complications such as anaemia due to ineffective erythropoiesis, iron overload, hypercoagulability and hormonal imbalances like hypogonadism, hypoparathyroidism, renal dysfunction etc.

METHODS

Our study was conducted in the Thalassemia Out Patient Department, Institute of Haematology & Transfusion Medicine at Medical College, Kolkata, over a span of 1 year 6 months. It was a cross sectional observational study of 30 patients of Non-Transfusion Dependant Thalassemia (NTDT) selected randomly as per the inclusion criteria. Thorough history taking and clinical examination were performed. Blood samples were tested for haemoglobin levels, RBC indices, fasting glucose, serum calcium, serum phosphate, SGPT, serum creatinine, TSH, FT4, FSH and LH (3 pooled samples at 30-minute-intervals), serum testosterone (in males) and serum ferritin. Ultrasonography and echocardiography were done. The data was analysed by standard statistical methods, using MedCalc (version 3.0) software. The correlation of different complications of NTDT with serum ferritin levels was done using Mann-Whitney U test. An alpha level of 5% has been taken i.e. any p value < 0.05 has been taken as significant

RESULTS

Serum ferritin levels were found to be quite high in the NTDT patients, with a range of 335.1 (min.) - 1300 (max.) ng/mL, with a mean serum ferritin level of 568.78 ng/mL and SD of 224.9, despite the fact that nearly 87% (26 out of 30) of the patients had received less than 10 transfusions in their lifetime. Our study showed that, delayed puberty, renal dysfunction, liver dysfunction and pulmonary hypertension were found to be significantly related to the serum ferritin levels (serving as a marker of liver iron concentration).

CONCLUSIONS

Thus, we conclude that despite requiring much fewer transfusions than transfusion dependant thalassemia patients, the NTDT patients do develop iron overload as well as different complications, some of which are significantly related to the liver iron overload. Knowledge of such complications could help to initiate chelation therapy at the appropriate time for NTDT patients, thereby reducing morbidity and improving their quality of life.

KEY WORDS

Non-Transfusion Dependent Thalassemia (NTDT), Ferritin