Primary AL amyloidosis is a plasma cell dyscrasia characterized by the deposition of monoclonal immunoglobulin light-chain protein. The latter forms insoluble fibrils with -pleated sheet configuration within a variety of tissues, resulting in severe organ dysfunction and poor outcome. In patients with primary AL amyloidosis, cyclic treatment with melphalan and prednisone improves by 2-fold medi...

primary localized amyloidosis of eyelid is a localized type of amyloidosis without evidence of systemic involvement. we report a 75-year-old man suffering of unilateral (left) ptosis due to upper eyelid mass with bony consistency. eye examination revealed upper lid mass attached to tarsus. no ocular infection or inflammation was found. pathologist reported diffuse distribution of eosinophilic h...

Lixelle is a direct hemoperfusion-type adsorption column that was developed to selectively eliminate beta2-microglobulin (beta2-m) from the circulating blood of patients with dialysis-related amyloidosis (DRA). The adsorbent in Lixelle comprises porous cellulose beads to which hydrophobic hexadecyl alkyl chain is covalently bound. One milliliter of wet Lixelle beads eliminates more than 1 mg of...

BACKGROUND The natural history of AA amyloidosis is typically progressive, leading to multiple organ failure and death. We analyzed the etiology as well as clinical and laboratory features of patients with biopsy-proven AA amyloidosis and evaluated the ultimate outcome. METHODS Seventy-three patients (24 female; mean age 41.85±15.89 years) were analyzed retrospectively. Demographic, clinical ...

The patient was a 66-year-old man who had undergone hemodialysis for 20 years. He had suffered from right hip pain for several months. He could stand, but his walking endurance was poor. Plain radiograph of the right hip showed osteoporotic change. T1-weighted (Fig. A) and TI inversion recovery MRI images (Fig. B) of bilateral hips showed low-signal-intensity thickened synovium (long arrows) ca...

background beta-2 microglobulin (b2m) is considered as a surrogate marker for middle molecule uremic toxins and a key component in dialysis-related amyloidosis. however, few studies have evaluated role of b2m in patients with chronic kidney disease (ckd). objectives the purpose of this study was to evaluate the association of plasma b2m level with some metabolic and cardiac performance factors ...