OBJECTIVE The diagnosis of amyloidosis still relies on biopsy, but there has been a growing demand for the development of a specific noninvasive diagnostic technique. Hepatocyte growth factor (HGF) acts on a variety of epithelial cells in multiple ways and is predominantly produced by mesenchymal cells and macrophages. In the present study, we measured the serum HGF level in patients with amylo...

OBJECTIVES We sought to evaluate the diagnostic performance of cardiovascular magnetic resonance imaging (CMRI) for detection of cardiac amyloidosis compared with endomyocardial biopsy (EMB) in a clinical routine setting. BACKGROUND For the clinical workup of heart failure with restrictive filling, pattern cardiac amyloidosis is an important differential diagnosis that is difficult to verify ...

Amyloidosis is a disease characterized by aberrant precursor molecules whose misfolded intermediate forms aggregate and are deposited as interstitial fibrils. The most common type of systemic amyloidosis is immunoglobulin light-chain amyloidosis (AL). Less common types of systemic amyloidosis are the transthyretin (ATTR) types caused by either mutant (hereditary) variants or wild-type ("senile ...

Cardiac amyloidosis is a manifestation of one of several systemic diseases known as the amyloidoses.1,2 This uncommon disease is probably underdiagnosed, and even when a diagnosis of amyloidosis of the heart is made, the fact that there are several types of amyloid, each with its unique features and treatment, is often unrecognized. This can lead to errors in management and in the information c...

INTRODUCTION Amyloidosis derived from transthyretin (TTR) molecules is typically caused by mutations of the TTR gene. METHODS We describe an elderly patient with a severe length-dependent polyneuropathy that unexpectedly proved to be caused by wild-type transthyretin amyloidosis. RESULTS The diagnosis was made by muscle biopsy, because no amyloid deposits were found in the biopsied nerve se...

Dynamic left ventricular outflow tract (LVOT) obstruction is classically seen in hypertrophic obstructive cardiomyopathy (HOCM). This can also be seen in cardiac amyloidosis. We describe a rare case of senile systemic amyloidosis with dynamic LVOT obstruction and concomitant three vessel coronary artery disease presenting with clinical and echocardiographic findings similar to those seen in HOC...

We report here on a rare case of primary AL hepatic amyloidosis associated with multiple myeloma in a 64-year-old woman. The patient was referred for evaluating her progressive jaundice and right upper quadrant pain. (18)F-fluorodeoxyglucose ((18)F-FDG) positron emission tomography (PET)/computed tomography (CT) showed diffusely and markedly increased (18)F-FDG uptake in the liver. Although the...

A patient with a 10 year history of monoclonal gammopathy of undetermined significance and Bence Jones proteinuria, and a 44 year history of ankylosing spondylitis, developed a nephrotic syndrome secondary to renal amyloidosis. Clinically the amyloidosis was ascribed to Bence Jones proteinuria rather than to the burnt out ankylosing spondylitis. However, histochemical and immunofluorescence sta...

Light chain amyloidosis has very rarely been reported in association with chronic lymphocytic leukemia (CLL). We reported on a 76-years-old female who presented with simultaneous kappa-restricted chronic lymphocytic leukemia (CLL) and a lambda-restricted multiple myeloma with plasma cells causing AL amyloidosis involving the heart. While monoclonal immunoglobulins occasionallyproduced by CLL ha...

Amyloidosis is characterized by extracellular deposition of abnormal protein, consisting of primary, secondary, hemodialysis-related, hereditary, senile and localized type. Primary amyloidosis is associated with monoclonal light chains. Secondary amyloidosis is associated with inflammatory, infectious, and neoplastic diseases. Amyloid deposition in the gastrointestinal tract can manifest the sy...