Paraplegia in a thalassaemic patient with short stature.

Extramedullary hematopoiesis (EMH) is a normal compensatory reaction that occurs in almost all chronic hemolytic anemia, especially in transfusion independent thalassemia intermedia, and can involve many organs or tissues, including the epidural space leading to spinal cord compression syndrome. We present a case of EMH in a 29 year old woman with thalassemia major, regularly transfused since the time of diagnosis (age 21 months), who presented with sudden muscle weakness, difficulty walking and maintaining the upright position. Magnetic Resonance Imaging (MRI) of the thoracic spine showed spinal cord compression secondary to extramedullary hematopoiesis in the spinal canal, leading to early therapy.

Vertebral collapse in thalassemia intermedia: case report.

Over the last few decades, the use of regular blood transfusions and adequate iron chelation beginning in the first years of life has modified the clinical picture and the natural course of thalassemia major. With the rise in the average age of these patients new problems have emerged, in particular bone disease: osteopenia, osteoporosis and the increased risk of fractures have become important causes of morbidity in a population whose longevity is continuously increasing. The Authors describe the case of a 41 year old patient affected by clinical thalassemia intermedia who presented with vertebral collapse after mild trauma.

Prospective evaluation of patient-reported outcomes during treatment with deferasirox or deferoxamine for iron overload in patients with beta-thalassemia.

Background: Iron chelation therapy (ICT) with deferoxamine (DFO), the current standard for the treatment of iron overload in patients with transfusion-dependent disorders such as beta-thalassemia, requires regular subcutaneous or intravenous infusions. This can lead to reduced quality of life and poor adherence, resulting in increased morbidity and mortality in iron-overloaded patients with beta-thalassemia. Deferasirox is an orally administered iron chelator that has been approved for use in the United States, Switzerland, and other countries.

Effects of acarbose in beta-thalassaemia major patients with normal glucose tolerance and hyperinsulinism.

The pathogenesis of diabetes in thalassaemia is complex and multifactorial. Understanding the sequence of abnormalities in the progression from normal glucose tolerance to impaired glucose tolerance may help in the formulation of ways to intervene in this process. In our study, we assessed the effects of acarbose, an alpha-glucosidase inhibitor, in five young adult thalassaemic patients with hyperinsulinism and normal oral glucose tolerance test (OGTT).