BACKGROUND In patients with thalassemia, chronic anemia causes bone marrow expansion and consequently skeletal manifestation in spine, skull, face and rib bones. OBJECTIVES We aimed to compare chest radiographic findings and facial bone deformity in patients with thalassemia major (TM) and intermedia. PATIENTS AND METHODS In this cross sectional study, 86 consecutive thalassemia patients re...

Spinal cord compression due to extramedullary hematopoiesis (EMH) is a rare complication of thalassemia and generally presents as paraparesis with sensory impairment. Complete paraplegia is extremely rare in EMH due to thalassemia although it is known to occur in polycythemia vera and sickle cell anemia. Treatment options mostly include surgery and/or radiotherapy. Whereas cases presenting with...

Extramedullary hematopoiesis is a common phenomenon in thalassemia. During the disease there are very rare occasions when compensatory hematopoietic tissue is located in the intraspinal epidural space, causing spinal cord compression. This complication requires urgent neurosurgical consideration and decision for further treatment. We present a case of thoracic spinal cord compression secondary ...

Background Thalassemia intermedia (TI) patients were shown to have significantly higher cardiac output and cardiac volumes with respect to thalassemia major (TM) patients. So, to compare biventricular parameters in TI patients with established ranges from TM may be misleading. The aim of this study was to establish the ranges for normal biventricular volumes and ejection fraction (EF) and for l...

Some hematological diseases can benefit from splenectomy for either diagnosis or treatment. When dealing with patients with thalassemia, splenectomy is a common therapeutic option, particularly indicated when the patient has increased transfusion demand, symptomatic splenomegaly or any signs of poor health, leucopenia, and thrombocytopenia.1 However, splenectomy can lead to thromboembolic event...

INTRODUCTION Extramedullary hematopoiesis occurs in approximately 15% of cases of thalassemia. Intracranial deposits of extramedullary hematopoiesis are an extremely rare compensatory process in intermediate and severe thalassemia. CASE PRESENTATION We present an unusual case of an intracranial extramedullary hematopoiesis with a choroid plexus origin in a 34-year-old Caucasian man with beta ...

UNLABELLED Thalassemias are the most common genetic disorder on a wordwide basis. β-thalassemia is a severe hemolytic anemia which results from genetic defects in the synthesis of the hemoglobin β-chain. Various endocrine abnormalities have been described in patients with thalassemia major. Endocrine disturbances have also been observed in patients with thalassemia intermedia (TI). In this stud...

BACKGROUND In the past two decades, a large-scale survey of hemoglobinopathies and thalassemia was carried out in mainland China, involving nearly one million people in 28 provinces. The incidences of hemoglobin (Hb) variants, alpha-thalassemia and beta-thalassemia were 0.33%, 2.64% and 0.66%, respectively. The chemical structural analysis identified 67 Hb variants. Among them, 20 are new varia...

B eta-thalassemias are heterogeneous autosomal recessive hereditary anemias characterized by reduced or absent b globin chain synthesis. The resulting relative excess of unbound a globin chains precipitate in erythroid precursors in the bone marrow, leading to their premature death and, hence, to ineffective erythropoiesis. b-thalassemia phenotypes are variable, ranging from the severe transfus...