THALASSAEMIA INTERMEDIA : AN UPDATE
<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 a...
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| Main Authors: | , , |
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| Format: | Article |
| Language: | English |
| Published: |
PAGEPress Publications
2009-06-01
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| Series: | Mediterranean Journal of Hematology and Infectious Diseases |
| Subjects: | |
| Online Access: | http://www.mjhid.org/article/view/4709 |
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| Summary: | <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> |
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| ISSN: | 2035-3006 |