La Revue de médecine interne
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Behçet disease is a multi-systemic complex vasculitis with unknown etiology characterized by different clinical involvements, including mucocutaneous, ocular, vascular, articular, neurological and gastrointestinal manifestations. Growing evidence supports that different phenotypes, characterized by clusters of co-existing involvements, can be distinguished. Namely, the vascular phenotype identifies a specific group of patients who suffer from recurrent inflammatory thrombosis and arterial involvement. ⋯ Arterial disease involves mostly pulmonary arteries and aorta and manifests mainly in the form of aneurysms. Glucocorticoids and immunosuppressant's are the recommended first-line treatments in vasculo-Behçet. Furthermore, randomized controlled trials are still needed to assess the role of adding anticoagulation to current standard therapy in venous thrombosis in Behçet's disease and to assess the role of anti-TNF alpha therapy in vasculo-Behçet.
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Chronic periaortitis (CP) is a rare disease characterised by the presence of a fibro-inflammatory tissue typically enveloping the abdominal aorta, the iliac arteries and, in some cases, the nearby structures, such as the ureters and the inferior vena cava. Imaging plays a key role in the diagnosis and follow-up: computed tomography and magnetic resonance imaging scans are used to define the extension of the pathological tissue, whereas fluorodeoxyglucose positron emission tomography is the gold standard to establish the degree of its metabolic activity. CP must be distinguished from secondary forms of periaortic infiltration, which include malignant, infectious, and drug-related aetiologies. This review focuses on the clinical aspects of CP and the differential diagnosis with secondary cases, and aims to provide the clinician with a guide through this challenging clinical approach.
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Alpha thalassemia-myelodysplastic syndrome (ATMDS) is one of the possible complications related to the genetic instability typical of clonal hemopoietic disorders such as myelodysplastic syndromes (MDS). Hemoglobin H acquisition, which is hemoglobin without alpha chains and with 4 beta chains is the hallmark of this disease. ⋯ The diagnosis of ATMDS should be considered in the presence of the association of MDS, microcytic anemia and marked blood smear abnormalities such as anisocytosis and poikilocytosis. A little less than 10% of all MDS are complicated by ATMDS.