@article{Taher_Musallam_Cappellini_2009, title={THALASSAEMIA INTERMEDIA : AN UPDATE}, volume={1}, url={https://www.mjhid.org/mjhid/article/view/2009.004}, abstractNote={<p class="MsoNormal" style="margin: 0cm 0cm 0pt; line-height: 200%; text-align: justify; mso-layout-grid-align: none;"><span style="font-size: 12pt; line-height: 200%; font-family: " lang="EN-US">Our understanding of the molecular and pathophysiological mechanisms underlying the disease process in patients with thalassaemia intermedia (TI) has substantially increased over the past decade. </span><span style="font-size: 12pt; color: #000000; line-height: 200%;">TI encompasses a wide clinical spectrum of beta-thalassaemia phenotypes. Some TI patients are asymptomatic until adult life, whereas others are symptomatic from as young as 2 years of age. A number of clinical complications commonly associated with TI are rarely seen in thalassaemia major, including extramedullary hematopoiesis, leg ulcers, gallstones, thrombosis and pulmonary hypertension. </span><span style="font-size: 12pt; color: #000000; line-height: 200%;">There are a number of options currently available for managing patients with TI, including transfusion therapy, iron chelation therapy, modulation of foetal haemoglobin production and haematopoietic stem cell transplantation. However, a</span><span style="font-size: 12pt; color: #000000; line-height: 200%;">t present, there are no clear guidelines for an orchestrated optimal treatment plan. </span></p>}, number={1}, journal={Mediterranean Journal of Hematology and Infectious Diseases}, author={Taher, Ali and Musallam, Khaled M. and Cappellini, Maria Domenica}, year={2009}, month={Aug.}, pages={e2009004} }