概 要IgG(λ)型 多 発性 骨髄腫 に合併 した びまん性肺胞 中隔 型肺 ア ミロ イ ド症 の1症 例 を報告 した.こ の まれ な多発 性骨 髄腫 にお け る肺 ア ミロイ ド症 の病 態 にっい て考 察 した.症 例.59才, 男性.主 訴,労 作時 呼吸 困難.胸 部X線 写真上,全 肺 野 にび まん性小 粒状陰 影 を認 め る.骨X線 像 では,左 上腕 骨 に打 ち抜 き像,骨 髄 中骨 髄腫細 胞24%,2.3g/dlのlg(x(λ)型M成 分,Bence Jones蛋 白陰性,肺 機 能検 査 ではDLCO著 減(%DLCO52%)を 示 した.肺 生検像 で は,単 ク ロ一 ン性形 質細 胞,す なわ ち骨 髄腫 細胞 の浸潤 と,肺 胞 壁,小 血管壁 の アミロイ ド沈着 を証明 した.メ ル フ ァラ ン,プ レ ドニ ゾロ ンの 治療 に ...

In secondary amyloidosis the kidney is one of the commonest sites in the body for the deposition of amyloid, and renal failure is a common sequel. In primary amyloidosis renal involvement is less common and renal failure exceptional. Only four cases of renal failure due to primary amyloidosis have been described in the literature. The individual patients of Christian (1932), Gerber (1934), and ...

AMYLOIDOSIS is a disease characterized by deposition of amyloid proteins in various body tissues. The natural history of primary amyloidosis is poorly understood, and the clinical diagnosis is often not made until the disease is far advanced. There is a high prevalence of conduction disturbances associated with this disease, and heart-related problems are a frequent cause of death for patients ...

Hepatic involvement in primary amyloidosis is an infrequent challenge to the hepatologist. Although usually asymptomatic, amyloidosis may have unusual manifestations. Liver biopsy is an important diagnostic tool for this condition. Herein, we report three cases of portal hypertension related to primary hepatic amyloidosis, one of them in the form of acute liver failure.

PRIMARY cerebral amyloidosis is characterized by deposition of amyloid within the cerebral tissue (e.g., core of neuritic plaques and core of plaques in spongiform encephalopathies) and the cerebral and meningeal blood vessel walls (amyloid or congophilic angiopathy) in the absence of systemic amyloidosis. Primary cerebral amyloidosis has been associated with normal brain aging changes" and wit...

This study was performed in real clinical practice settings. Our paper describes two case studies which show the difficulty of differential diagnosis monoclonal gammopathies, multiple myeloma associated with AL-amyloidosis and primary AL-amyloidosis.

BACKGROUND Primary localized cutaneous amyloidosis (PCA) is a relatively rare condition characterized by amyloid deposition in dermis without systemic involvement. Although, histopathological examination of the lesion reveals amorphous eosinophilic deposits in papillary dermis examination of congo red stained slides under polarized light will give definitive diagnosis AIMS To study the clinic...

position of insoluble fibrillar proteins in various organs [1]. In humans, more than 23 different and unrelated proteins are known to form amyloid fibrils [2]. Amyloidosis is divided into primary (i. e. idiopathic) and secondary amyloidosis (i. e. associated with chronic inflammatory conditions, and infectious and neoplastic disorders) [1]. Primary amyloidosis is extremely rare in the gastroint...

Although amyloidosis of the respiratory tract is well recognized, pleural involvement is very rare with only two cases being reported in the past. We report a case of primary amyloidosis with pleural effusion and suggest that pleural involvement and pleural effusion be added to the classification of pulmonary amyloidosis, and that amyloidosis be added to the list of causes of a pleural effusion.