Small plasma cell clones can cause big problems2; this could be the outline for AL amyloidosis. AL amyloidosis is the most common form of systemic amyloidosis, a protein misfolding disease that is characterized by the failure of the amyloidogenic monoclonal light chain to adopt a soluble conformation. This misfolding leads to the formation of a precursor protein that tends to form highly organi...

Amyloidosis is a rare disorder characterised by the deposition of abnormal protein fibrills in various tissues and organs.In context multiple myeloma,a plasma cell dyscrasia amyloidosis can occur as distinct complication, referred to myeloma associated amyloidosis.

BACKGROUND Amyloid deposition in heart is a common occurrence in systemic amyloidosis. But localised valvular amyloid deposits are very uncommon. It was only in 1922 that the cases of valvular amyloidosis were reported. Then in 1980, Goffin et al reported another type of valvular amyloidosis, which he called the dystrophic valvular amyloidosis. We report a case of aortic valve amyloidosis which...

BACKGROUND Patients with light chain (AL) amyloidosis who present with severe heart failure due to cardiac involvement rarely survive more than 6 months. Survival after cardiac transplantation is markedly reduced due to the progression of amyloidosis. Autologous stem-cell transplantation (ASCT) has become a common therapy for AL amyloidosis, but there is an exceedingly high treatment-related mo...

OBJECTIVES To examine dermatologic adverse effects of lenalidomide in patients with amyloidosis and multiple myeloma and to determine whether the adverse effects are different when lenalidomide is used alone compared with when it is used in combination with dexamethasone. DESIGN Retrospective review of medical records. SETTING Tertiary referral center. PATIENTS Seventy-five patients with ...

In immunoglobulin light chain (AL) amyloidosis, amyloid fibril deposits derived from immunoglobulin light chains produced by a clonal plasma cell dyscrasia accumulate in tissues and damage vital organs. Treatment regimens used in multiple myeloma can be effective in AL amyloidosis; however, patients with this disease often tolerate these regimens poorly because of multisystem organ dysfunction....

Apolipoprotein A-II (apoA-II) is the second major apolipoprotein following apolipoprotein A-I (apoA-I) in HDL. ApoA-II has multiple physiological functions and can form senile amyloid fibrils (AApoAII) in mice. Most circulating apoA-II is present in lipoprotein A-I/A-II. To study the influence of apoA-I on apoA-II and AApoAII amyloidosis, apoA-I-deficient (C57BL/6J.Apoa1⁻/⁻) mice were used. Apo...

A nonimmunoglobulin protein with the molecular weight of 9,145 (protein AS) has been shown to be a principal component of the amyloid fibrils in different clinical types of amyloidosis. A protein component, antigenically closely related to protein AS, was detected in human sera. The protein AS-related component (protein ASC) was found in the sera of many groups of patients, including 48 out of ...

High-dose chemotherapy with autologous stem cell transplantation (SCT) is widely used as a treatment for systemic AL amyloidosis, but its efficacy has not been proved and it has substantial toxicity in this setting. We report here the outcome of 92 patients evaluated at the UK National Amyloidosis Centre who underwent SCT for AL amyloidosis between 1994 and 2004 in various British centres. Medi...

objective(s):familial mediterranean fever (fmf), an inherited autosomal recessive disorder, is frequently present among individuals of mediterranean origin. differences in the clinical manifestations of fmf between different ethnic groups have been documented. the aim of the present study was to determine the most common characteristics of fmf and the relationship between clinical findings and ...