Anti-αIIbβ3immunization in Glanzmann thrombasthenia: review of literature and treatment recommendations

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Glanzmann thrombasthenia

Glanzmann thrombasthenia (GT) is a rare autosomal recessive bleeding syndrome affecting the megakaryocyte lineage and characterized by lack of platelet aggregation. The molecular basis is linked to quantitative and/or qualitative abnormalities of alphaIIb beta3 integrin. This receptor mediates the binding of adhesive proteins that attach aggregating platelets and ensure thrombus formation at si...

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Glanzmann thrombasthenia in a neonate.

Glanzmann thrombasthenia is a qualitative platelet function disorder manifested by skin bleeds, epistaxis, gingival bleeding, gastrointestinal hemorrhage, hematuria, hemarthrosis, intracranial hemorrhage and visceral hematomas. We report a six day old newborn presenting with hematuria following suprapubic aspiration, who was diagnosed as Glanzmann thrombasthenia. We believe it to be the younges...

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Glanzmann Thrombasthenia: A Clinicopathological Profile.

OBJECTIVE To describe the clinical presentation of patients with Glanzmann's thrombasthenia (GT) and evaluate their diagnostic, clinical, and laboratory parameters including platelet aggregometry. STUDY DESIGN Descriptive study. PLACE AND DURATION OF STUDY Department of Hematology and Blood Transfusion, The Children Hospital and Institute of Child Health, Lahore, from January 2006 to Decemb...

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Patients With Glanzmann Thrombasthenia Lacking Platelet Glycoprotein

Background—Platelets have been suggested to play a role in the early development of atherosclerosis. As one test of this hypothesis, we assessed whether patients with Glanzmann thrombasthenia who lack platelet glycoprotein IIb 3 (GPIIb/IIIa) complexes or both IIb 3 and the more ubiquitous v 3 cell membrane complexes are protected from development of atherosclerosis. Methods and Results—Seven pa...

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Panner’s disease: literature review and treatment recommendations

PURPOSE To determine the most up-to-date theory on the aetiology of Panner's disease, to form a consensus on the assessment of radiographs and to evaluate clinical outcome in order to summarise the best available evidence for diagnosis and treatment. METHODS A review of studies to date on Panner's disease. Studies were eligible if: (1) the study provided criteria for defining Panner's disease...

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ژورنال

عنوان ژورنال: British Journal of Haematology

سال: 2018

ISSN: 0007-1048

DOI: 10.1111/bjh.15087