Format

Send to

Choose Destination
See comment in PubMed Commons below
Mediterr J Hematol Infect Dis. 2009 Aug 29;1(1):e2009004. doi: 10.4084/MJHID.2009.004.

Thalassaemia intermedia: an update.

Author information

1
Department of Internal Medicine, Hematology-Oncology Division, American University of Beirut Medical Centre, Beirut, Lebanon.

Abstract

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. 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. 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, at present, there are no clear guidelines for an orchestrated optimal treatment plan.

PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Catholic University in Rome, Institute of Hematology Icon for PubMed Central
    Loading ...
    Support Center