Recurrent Pulmonary Embolism and Pulmonary Hypertension in a Patient with β-Thalassemia Intermedia

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Thalassemia Associated Pulmonary Hypertension

Cardiac disease is the main cause of death in both forms of thalassemia; thalassemia major (TM) and thalassemia intermedia (TI). Pulmonary hypertension (PH) is one of the cardiopulmonary morbidities with high mortality that, if not treated, may trigger right-sided heart failure and premature death. PH is defined as a mean pulmonary artery pressure of ≥25 mmHg at rest or ≥30 mmHg during exercise...

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Center for Human Genetics for outstanding cytogenetic and FISH experiments, Dr L. Knoops (Cliniques Universitaires Saint-Luc, Université Catholique de Louvain and Ludwig Institute for Cancer Research, Brussels) for discussions, and Katrien Van Roosbroeck (KU Leuven, Leuven) for providing SEC31A cDNA. They also thank Novartis (Vilvoorde, Belgium) for kindly supplying ruxolitinib. This work was s...

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Pulmonary hypertension (PH) is frequent among patients with β-thalassemia intermedia (TI) and β-thalassemia major (TM) (1). Almost 60% of all TI patients develop PH (2). However, no randomized controlled trials have evaluated this condition-specific treatment options. Recent guidelines for the treatment of PH offer no specific recommendations for these patients; moreover, the classification of ...

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ژورنال

عنوان ژورنال: Journal of Blood & Lymph

سال: 2018

ISSN: 2165-7831

DOI: 10.4172/2165-7831.1000205