Abstract
系统性轻链型淀粉样变性是一种浆细胞疾病,淀粉样蛋白异常沉积于细胞外基质,造成沉积部位组织和器官损伤,可累及肾脏、心脏、肝脏、皮肤软组织、神经和肺等器官。本病预后不良,早诊早治至关重要,但因其临床表现多样,早期极易漏诊、误诊。本文报道1例以皮肤角质囊肿为首发症状的系统性轻链型淀粉样变性,并进行文献复习,以期提高临床、病理医师对此病的认识。.
MeSH terms
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Amyloidosis* / diagnosis
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Amyloidosis* / metabolism
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Amyloidosis* / pathology
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Epidermal Cyst / metabolism
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Epidermal Cyst / pathology
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Female
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Humans
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Immunoglobulin Light Chains / metabolism
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Immunoglobulin Light-chain Amyloidosis / diagnosis
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Immunoglobulin Light-chain Amyloidosis / metabolism
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Immunoglobulin Light-chain Amyloidosis / pathology
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Male
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Middle Aged
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Multiple Myeloma* / complications
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Multiple Myeloma* / diagnosis
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Multiple Myeloma* / pathology
Substances
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Immunoglobulin Light Chains