Case: Breast Amyloidosis Detected With 3D Mammography Case: Breast Amyloidosis Detected With 3D Mammography
Review the imaging characteristics of breast amyloidosis in this unusual case. How should definitive diagnosis be made?Applied Radiology
ConclusionNon-invasive tests for the detection of cardiac amyloidosis, like myocardial scintigraphy with bone seeking tracers, can play a major role in the diagnosis progression and therapeutic management of patients with cardiac amyloidosis.
Familial Mediterranean fever (FMF) is a genetically inherited autoinflammatory disorder characterized by inflammatory attacks and may result in amyloidosis as a severe complication. Elevation of acute phase reactants, including leukocytosis, is seen during attack periods. Here we describe a 13-year-old female patient with a very rare clinical presentation of FMF, who would experience FMF attacks when she did not regularly take her colchicine. During these attacks she had leukopenia and neutropenia instead of leukocytosis. The leukocyte count returned to normal when she continued the medication and avoided attacks. Ethnicit...
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AbstractIn this issue of JNC, BW Spery and Coll report a retrospective analysis of 57 patients with transthyretin-related amyloidosis (ATTR) in an advanced phase of the disease who underwent 99mTechnetium-pyrophosphate (99mTcPYP) scintigraphy. Although relatively small and “negative,” the study is relevant since it broadens our knowledge on the uptake of “bone tracers” in ATTR and contributes to understand the limitations of the clinical use of scintigraphy in this disease. The paper raises, directly or indirectly, at least three questions: To what extent are the different bone tracers interchangeab...
Prognostic significance of circulating plasma cells by multi-parametric flow cytometry in light chain amyloidosis, Published online: 21 February 2018; doi:10.1038/s41375-018-0063-7Prognostic significance of circulating plasma cells by multi-parametric flow cytometry in light chain amyloidosis
Depth of organ response in AL amyloidosis is associated with improved survival: grading the organ response criteria, Published online: 20 February 2018; doi:10.1038/s41375-018-0060-xDepth of organ response in AL amyloidosis is associated with improved survival: grading the organ response criteria
ConclusionsResponses to daratumumab and combinations in patients with advanced MM, particularly with extramedullary disease, are low and short lived, stressing the administration of this agent should be early in the course of the disease.This article is protected by copyright. All rights reserved.
We examined the prognostic use and survival association of serum TTR (transthyretin) concentration in ATTRwt. Methods and Results: Patients with biopsy-proven ATTRwt were retrospectively identified. Serum TTR, cardiac biomarkers, and echocardiographic parameters were assessed at baseline and follow-up evaluations. Statistical analyses included Kaplan–Meier method, Cox proportional hazard survival models, and receiver-operating characteristic curve analysis. Median serum TTR concentration at presentation was 23 mg/dL (n=116). Multivariate predictors of shorter overall survival were decreased TTR, left ventricular eje...
In this study, we retrospectively evaluated 11 pediatric colchicine-resistant FMF patients who were t reated with canakinumab. Three of the patients had amyloidosis and two had uveitis. Based on our results, we suggest that canakinumab may be a safe and effective therapy in patients who are resistant to colchicine and even in the patients with amyloidosis. We also suggest that canakinumab might be a safe option for the patients with uveitis.