Infiltrative heart diseases are caused by a heterogeneous group of disorders; amyloidosis and sarcoidosis are two frequent causes of myocardial infiltration, which differ in clinical and biological outcome and treatment issues. The presence of high levels of angiotensin-converting enzyme (ACE) in a patient with infiltrative heart disease may increase suspicion of sarcoidosis. Nevertheless, no m...

Systemic amyloidosis of amyloid light chain associated protein (AL), also called primary amyloidosis, frequently involves the liver, but rarely causes clinically apparent liver disease. The more common presentation is with acute renal failure. Hepatomegaly and mild elevation of alkaline phosphatase are the most common clinical and biochemical findings, respectively. We report a case of systemic...

Nonhematologic malignancies are rarely reported to be associated with AA amyloidosis. Although the association between renal cell carcinoma and systemic AA amyloidosis has been established, the evidence linking pulmonary cancer to AA amyloidosis is scarce. Here, a case of biopsy-proven renal AA amyloidosis complicated with nephrotic syndrome associated with lung carcinoma is reported.

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 ...

Hereditary fibrinogen Aα-chain amyloidosis (Aα-chain amyloidosis) is a type of autosomal dominant systemic amyloidosis caused by mutations in fibrinogen Aα-chain gene (FGA). Patients with Aα-chain amyloidosis have been mainly reported in Western countries but have been rarely reported in Asia, with only five patients with Aα-chain amyloidosis being reported in Korea, China, and Japan. Clinicall...

In a general hospital, 391 consecutive necropsies in which at least seven organs were available, were examined retrospectively by polarizing microscopy of Congo-red-stained sections for the presence of local amyloid deposits.Non-systemic microdeposits of amyloid were encountered in 72 cases, an overall incidence of 18.4%. They were usually small and frequently detectable only by virtue of polar...

Primary systemic amyloidosis (PSA) mostly occurs in the setting of plasma cell dyscrasias.[1] It is difficult to diagnose as it usually presents with nonspecific symptoms.[2] Mucocutaneous manifestations occur in 29%–40% of cases and provide important diagnostic clues.[3] We report an atypical case where an unusually extensive cutaneous involvement provided the first sign of a widespread multio...

Studies of lysozyme have played a major role over several decades in defining the general principles underlying protein structure, folding, and stability. Following the discovery some 10 years ago that two mutational variants of lysozyme are associated with systemic amyloidosis, these studies have been extended to investigate the mechanism of amyloid fibril formation. This Account describes our...

Primary cutaneous amyloidosis is limited to the skin without involving any other tissue. Nodular amyloidosis is rare, and atrophic nodular cutaneous amyloidosis is even rarer. We describe the fourth case of atrophic nodular cutaneous amyloidosis by searching PubMed databases. A 52-year-old female presented to our hospital with a 2-year history of orange papules and nodules without subjective sy...