Systemic amyloid light chain (AL) amyloidosis (previously known as primary amyloidosis) is a multisystem disease characterized by the extracellular deposition of fibrils composed of immunoglobulin light chains within various organs. AL amyloidosis is associated with different types of monoclonal plasma cell dyscrasias, including multiple myeloma and other monoclonal gammopathies.1 Cardiac invol...

AA amyloidosis is one of the principal causes of morbidity and mortality in captive cheetahs (Acinonyx jubatus), which are in danger of extinction, but little is known about the underlying mechanisms. Given the transmissible characteristics of AA amyloidosis, transmission between captive cheetahs may be a possible mechanism involved in the high incidence of AA amyloidosis. In this study of anim...

Primary localized cutaneous amyloidosis (PLCA) is caused by the extracellular deposition of amyloid material in the skin without other cutaneous or systemic organ involvement. PCLA is classified into lichen, macular,and nodular amyloidosis. Macular amyloidosis and lichen amyloidosis are named as biphasic amyloidosis when they are concurrently seen in a patient. The treatment of this disease is ...

Cardiac involvement occurs frequently in primary amyloidosis and is associated with heart failure hospitalizations and poor survival. The initial presentation of the disease may be misleading, resulting in under-diagnosis of cardiac amyloidosis and late initiation of treatment. We present a case of cardiac amyloidosis initially misdiagnosed as hypertrophic cardiomyopathy and we discuss the key ...

Background Systemic amyloidosis is a disorder characterized by extracellular deposition of different insoluble protein fibrils in various organs leading to organ dysfunction. Cardiac involvement is associated with limited survival. We aimed to use cardiac magnetic resonance imaging (CMR) to identify structural and functional alterations related to the different forms of amyloidosis, e. g. hered...

Globular amyloidosis is a very infrequent amyloidosis subtype, characterized by the deposition of rounded bodies of protein, occasionally perivascular instead of the usual linear deposits. The most frequently affected organ is the liver, although other organs can also been involved. To date, only eight cases (1-3) of globular amyloidosis of the gastrointestinal tract have been described. Clinic...

Introduction FMF is associated with pulmonary hypertension(PH) due to amyloidosis. However, clinically overt PH with right-sided heart failure remains a rare event limited to few patients with pulmonary amyloidosis secondary to FMF. We report two cases of FMF patients, with and without amyloidosis, who experienced PH with rightsided heart failure. To our knowledge, this is the first case report...

Examination of abdominal subcutaneous fat aspirates is a practical, sensitive and specific method for the diagnosis of systemic amyloidosis. Here we describe the development and implementation of a clinical assay using mass spectrometry-based proteomics to type amyloidosis in subcutaneous fat aspirates. First, we validated the assay comparing amyloid-positive (n=43) and -negative (n=26) subcuta...

Amyloidosis is characterized by the deposition of misfolded protein in various organs. The diagnosis of amyloidosis is based on the detection of amyloid deposits by Congo red stain under polarized microscopy. This study was done to investigate C4d deposition in amyloidosis and to determine whether C4d staining can be used as a new diagnostic tool for amyloidosis. This retrospective study includ...

OBJECTIVE To determine whether or not the use of colchicine decreases the risk of amyloidosis among Armenian patients with familial Mediterranean fever (FMF). SUBJECTS AND METHODS The study included 99 Armenian patients from the Center of Medical Genetics database with genetically ascertained FMF; 33 had renal amyloidosis and 66 were randomly selected control patients without renal amyloidosi...