Background Transthyretin (TTR) amyloidosis is a progressive and fatal systemic disorder caused by misfolded TTR monomers that cumulatively deposit in the heart, peripheral nerves and other organ systems. TTR Amyloidosis-associated cardiomyopathy (ATTR-CM), caused by TTR amyloid infiltration of the myocardium and conduction system, results in a restrictive cardiomyopathy associated with atrial a...

Background: Systemic amyloidosis is characterized by amyloid deposition that can involve virtually any organ. Splenic and hepatic occurs in certain types, some patients but not others, may influence prognosis treatment. SAP (serum P component) scintigraphy uniquely able to identify quantify the liver spleen, thus informing clinical management, it only available 2 centers globally. The aims of t...

Gastrointestinal amyloidosis (GIA), a protein deposition disorder, represents a complex common pathway that encompasses multiple etiologies and presentations. It represents a significant diagnostic and treatment challenge. The disease results from the deposition of insoluble extracellular protein fragments that have been rendered resistant to digestion. GIA can be acquired or genetic, and most ...

BACKGROUND Central nervous system (CNS) complications are increasingly noted in liver transplanted (LTx) hereditary transthyretin amyloid (ATTRm) amyloidosis patients; this suggests that the increased survival allows for intracranial ATTRm formation from brain synthesized mutant TTR. However, atrial fibrillation (AF), a recognised risk factor for ischemic CNS complications, is also observed aft...

Primary systemic amyloidosis is a rare entity. We report a case of primary systemic primary amyloidosis, an elderly male presented with generalized weakness since 6 months. Clinical examination revealed typical waxy lesions in the periorbital area with macroglossia. Serum electrophoresis was normal, however, bone marrow examination showed increased plasma cell with a good number of both binucle...

A 28-year-old man presented with a history of hyper-IgD and periodic fever syndrome (HIDS), which had resulted in systemic AA amyloidosis. He had evidence of amyloid deposition in the kidney, liver and spleen. A living donor kidney transplant in 2005 failed after 6 years due to recurrent amyloid in the transplant kidney. A trial of tocilizumab, an anti-IL-6 monoclonal antibody, started in July ...

Amyloidoses represent an inhomogeneous group of diseases characterized by extracellular deposition of amyloid fibrils. AA amyloidosis is a serious life-threatening complication of chronic rheumatic diseases responsible for increased mortality due to organ failure or infection. The main component of amyloid is the serum amyloid A precursor protein (SAA) produced by the liver as an acute phase pr...

The case presented in this report is one of 45-year-old female with dysphagia and swelling in the neck since 2 months, breathlessness, history of inability to open the mouth and course of events resembled scleroderma and diagnosis of primary systemic amyloidosis could not be established until autopsy. At autopsy skin, oesophagus, salivary glands, tongue and all the parenchymal organs like splee...

Primary systemic amyloidosis is a rare disease. It primarily involves kidney, heart, peripheral nerves and liver. Intracutaneous hemorrhage manifesting in the form of petechiae, purpura and ecchymoses due to infiltration of blood vessel walls by amyloid deposits are the most common skin lesions. We report a case of primary systemic amyloidosis with multiple, non-itchy, papular lesions in lower ...

We hereby report a 79-year-old Iranian man presenting with nail dystrophy and subsequent development of purpuric and ecchymotic plaques, hemorrhagic bullae, and infiltrated papules on the head, neck and trunk. Histological examination of the gingiva, bone marrow aspiration, and biopsy confirmed the diagnosis of primary systemic amyloidosis. In this case, nail dystrophy was the presenting sign o...