Objective To retrospectively investigate the clinical manifestations of systemic amyloid light-chain (AL) amyloidosis in Japanese patients and the treatment strategy for the condition. Methods We conducted a survey of Japanese AL amyloidosis patients, who were treated between January 1, 2012, and December 31, 2014. Results A total of 741 AL amyloidosis patients were included in this study (436 ...

Amyloidosis has been classified by Symmers (1956) into (1) generalized secondary amyloidosis; (2) generalized primary amyloidosis ; and (3) localized amyloidosis. Involvement of the respiratory tract by amyloid material in the generalized systemic form of the disorder is not as rare as is commonly supposed. In amyloidosis associated with a recognized predisposing disease the incidence is at lea...

Domino liver transplantation, in which the liver from a patient with transthyretin-mediated amyloidosis (ATTR) is transplanted into another patient, is a well-established procedure. The figure is from 2 unrelated cases of liver transplant receptors from donors with ATTR who developed clinical polyneuropathy with weakness and paresthesia in lower extremities some years after the liver transplant...

The shortage of organs forces coordinators to seek new forms of generating organs for transplantation of the increasing numbers of patients on waiting lists. A recent technique called sequential transplant or domino liver transplant (DLT) allows the transplantation of a patient with chronic liver disease by implantation of a full-size liver derived from a patient with familial amyloidosis polyn...

Spongiform encephalopathies have been reported to be transmitted by blood transfusion even prior to the clinical onset. Experimental AA-amyloidosis shows similarities with prion disease and amyloid-containing organ-extracts can prime a recipient for the disease. In this systemic form of amyloidosis N-terminal fragments of the acute-phase reactant apolipoprotein serum amyloid A are the main amyl...

TTR familial amyloidosis is a systemic disease, with a frequent cardiac involvement For clinical reasons – related to its prolonged latency and historical reasons – as the first cases where diagnosed by neurologists, in young patients who died from neurological disease before cardiac complications occurredfamilial cardiac amyloidosis (TTR-FAC) has been and still remains underestimated. It is kn...

We report this case of secondary amyloidosis associated with Castleman's disease. A 51-year-old man presented with systemic symptoms of generalized weakness, fatigue, unintended weight loss, anorexia and progressively worsening abdominal distension. On examination he was found to have an indurated right-sided submandibular mass and tense ascites. He was found to have multiorgan dysfunction with...