Background Patients with thalassemia major and intermedia are susceptible to osteopenia and osteoporosis. The mechanism of osteoporosis in these patients is multifactorial. Transfusion related iron overload in endocrine organs leads to impaired growth hormone secretion, diabetes mellitus, hypothyroidism, hypoparathyroidism, lack of sex steroids and vitamin D deficiency that contribute t...

Deniz Tuğcu1, Zeynep Karakaş2, Müge Gökçe1, Leyla Ağaoğlu2, Ayşegül Ünüvar2, Ebru Sarıbeyoğlu2, Arzu Akçay1, Ömer Devecioğlu2 1Kanuni Sultan Süleyman Education and Research Hospital, Clinic of Pediatric Haematology-Oncology, İstanbul, Turkey 2İstanbul University, İstanbul Faculty of Medicine, Department of Pediatric Haematology-Oncology, İstanbul, Turkey Thalassemia Intermedia and Acute Lymphob...

Heart disease is the leading cause of mortality and one of the main causes of morbidity in beta-thalassemia. Patients with homozygous thalassemia may have either a severe phenotype which is usually transfusion dependent or a milder form that is thalassemia intermedia. The two main factors that determine cardiac disease in homozygous β thalassemia are the high output state that results from chro...

Introduction: Thalassemia is common in Iran. Appropriate therapy for this disease includes a regular blood transfusion and chelation therapy. However, patients will inevitably confront with side effects, particularly iron overloads in critical organ including heart, ductless glands and liver. This study tries to determine the prevalence of diabetes mellitus in transfusion dependent β thalassemi...

Extramedullary haemopoiesis causing spinal cord compression is a rare manifestation of thalassemia. We describe a 17 year old male with thalassemia intermedia who presented with progressive paraplegia and sphincter disturbance. Magnetic resonance imaging revealed an epidural lesion extending from T5 to T8 compressing the spinal cord. The patient recovered completely after surgical decompression...

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...