Prevalence and Risk Factors of Complications in Patients with Thalassemia Intermedia

BACKGROUND

Thalassemia intermedia (TI) or Non-transfusion dependent thalassemia (NTDT) is a mild form of thalassemia and does not require regular transfusion. Many complications can be found in this disease and different from thalassemia major(TM). Most studies mainly focus on beta thalassemia and lack of study in alpha group. This study aims to elucidate the prevalence of complications and identify predictive factors affecting complication of both alpha and beta NTDT patients.

METHODS

We retrospectively reviewed NTDT patients who attended our clinic from 1 January 2012 to 31 December 2013. Medical record was reviewed for complication and clinical data.

RESULTS

One hundred NTDT patients were included in this study, female (60%), median age 38 years (19-78). Alpha thalassemia was the majority population (54%). Interestingly, overall complications were found in 83%. The three most common were extramedullary hematopoiesis (47%), cholelithiasis (35%) and abnormal liver function (29%). The others were endocrinopathy (29%), osteoporosis (17%), pulmonary hypertension (14%), cardiomyopathy (11%), thrombosis (4%), and leg ulcer (2%), respectively. The mean ferritin level was 1,563.46 ng/ml. Forty four and seventy six percent of patients had ferritin level more than 2,500 and 800 ng/ ml, respectively.

The significant risk factors affecting complications in extramedullary hematopoiesis were female with odd ratio 2.76 (95% CI 1.046-7.294, p= 0.040) and hemoglobin level below 8 g/dL with odd ratio 3.08 (95% CI 1.113-8.521, p= 0.03) and the significant risk factors affecting complications in osteoporosis were female with odd ratio 7.64 (95% CI 1.514-38.604, p= 0.014) and age more than 40 years with odd ratio 4.66 (95% CI 1.313-16.506, p= 0.017). Iron overload (ferritin > 800 ng/ml) was the only risk factor for abnormal liver function from this study with odd ratio 3.79 (95% CI 1.033-13.919, p= 0.035), but trend to be statistical significant in other complications. Three patients were death and all of them died from sepsis.

CONCLUSION

Alpha thalassemia is the common type of NTDT in our study which is different from previous study. The complication in NTDT is also different from TM. The most common complications were extramedullary hematopoiesis, cholelithiasis and abnormal liver function. The prevalence of iron overload was high in these patients. Thus, regular iron monitoring and early investigation for complication detecting were necessary in this group of patients.

Figure 1

View largeDownload slide

Figure 1

View largeDownload slide

Close modal