The Netherlands journal of medicine
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Monoclonal gammopathy of renal significance (MGRS) includes all kidney disorders caused by a monoclonal protein (M-protein) secreted by a small plasma cell clone or other B-cell clones in patients who do not meet the diagnostic criteria for multiple myeloma or other B-cell malignancies. The underlying disorder in patients with MGRS is generally consistent with monoclonal gammopathy of undetermined significance (MGUS). MGRS-associated kidney disorders are various and the list is still expanding. ⋯ A combined haematologic and nephrologic approach is crucial to establish the causative role of the M-protein in the pathogenesis of kidney disease. Clone-directed therapy, which may include autologous stem cell transplantation in eligible patients, often results in improved outcomes. In this review, we discuss the histopathologic classification of MGRS lesions, provide a renal and haematologic diagnostic workup, discuss treatment options for MGRS, and introduce a Benelux MGRS Working Group.
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In the past decade, 18F-fluorodeoxyglucose positron emission tomography combined with computed tomography (18F-FDG PET-CT) scans have been increasingly implemented in the diagnostic process of several haemato-oncological conditions. Accurate assessment of bone marrow activity observed on 18F-FDG PET-CT is crucial for a correct diagnostic conclusion, subsequent treatment decision, and follow-up strategies. By systematically considering the arguments of the level of 18F-FDG uptake, distribution pattern, coinciding changes of the bone structure, and the clinical context, interpretation and validity may improve. This review aims to give a comprehensive overview of the different patterns of 18F-FDG uptake on PET/CT in common benign, clonal, and malignant haematological conditions, accompanied by illustrative cases.
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Outcomes of vascular surgery for patients with primary antiphospholipid syndrome (APS) presenting with acute limb ischaemia (ALI) are poor, with a high rate of postoperative arterial thrombosis and limb amputation. A primary antiphospholipid syndrome 42-year-old male patient presented with acute limb ischaemia. Timely endovascular thrombectomy successfully prevented irreversible tissue damage but failed to maintain this due to recurrent thrombosis. ⋯ Two weeks post-discharge, thrombotic arterial reocclusion led to readmission and repeated management. Following successful reperfusion, intensive immunosuppressive therapy and anticoagulant agents ensured that the patient was free from recurrent events during the next eight months. This case highlights the combination of endovascular thrombectomy and intensive plasma exchange for limb salvage in such cases.