BACKGROUND Beta thalassemia is one of the most common genetic disorders in the world. The aim of this study was to determine the frequency, characteristics, and pattern of malignancies in patients with beta thalassemia major (BTM) and beta thalassemia intermedia (BTI) in Iran. METHODS We conducted a multicenter study via a retrospective chart review of patients with BTM and BTI between 2002 a...

Pulmonary hypertension (PH) is frequent among patients with β-thalassemia intermedia (TI) and β-thalassemia major (TM) (1). Almost 60% of all TI patients develop PH (2). However, no randomized controlled trials have evaluated this condition-specific treatment options. Recent guidelines for the treatment of PH offer no specific recommendations for these patients; moreover, the classification of ...

Moyamoya syndrome (MMS) is a progressive disorder. We report a 19-year-old boy with beta-thalassemia who presented with a left hemiparesis. Brain MRI showed old middle cerebral artery and left frontal subcortical white matter infarcts. Brain magnetic resonance angiography and digital subtraction angiography revealed occlusion of the bilateral internal carotid arteries with a rich network of bas...

BACKGROUND Patients with thalassemia may complain of numbness and weakness of the lower extremities. The aim of the study was to document whether these patients suffer from a polyneuropathy and to determine any contributing factors for the development of neuropathy. PATIENTS AND METHODS We examined 30 patients with thalassemia major and intermedia, clinically and electrophysiologically. We co...

Mutations in human Krüppel-like factor 1 (KLF1) have recently been reported to be responsible for increased fetal hemoglobin (HbF) and hemoglobin A2 (HbA2). As increased HbF and HbA2 levels are important features of β-thalassemia, we examined whether there is any relationship between KLF1 mutation and β-thalassemia in China. For this, we first studied the incidence of KLF1 mutations in two Chin...

Mutations in human Krüppel-like factor 1 (KLF1) have recently been reported to be responsible for increased fetal hemoglobin (HbF) and hemoglobin A2 (HbA2). Because increased HbF and HbA2 levels are important features of β-thalassemia, we examined whether there is any relationship between KLF1 mutation and β-thalassemia in China. To do this, we first studied the incidence of KLF1 mutations in 2...

BACKGROUND The technology to recognize nucleated red blood cells (NRBC) automatically has only recently been developed. Modern hematology analyzers allow for rapid and accurate NRBC counts. The goal of our study was to evaluate NRBC counts and the concentrations of serum transferrin receptor (sTfR) in patients affected by different thalassemia syndromes and hereditary spherocytosis. We wished t...

PURPOSE To investigate the efficiency of partial radiofrequency ablation of the spleen in patients with thalassemia major and intermedia. MATERIALS AND METHODS Partial radiofrequency ablation of the spleen was performed in 19 thalassemic patients (10 females, nine males) with a mean age of 12.7 years (age range, 7-30 years). This group of patients consisted of 11 patients with thalassemia maj...

BACKGROUND Thalassemia is the most common inherited disease in southern China. However, this disorder is usually ignored by Jiangxi provincial health system and government due to lack of epidemiological data. MATERIALS AND METHODS A total of 9489 samples from Hakka Han and Gan-speaking Han in three geographical areas of Jiangxi Province were analyzed for both complete blood cell (CBC) count a...