Amyloidosis with plasma cell dyscrasia. An overlooked caused of adult onset sensorimotor neuropathy.
Amyloidosis with plasma cell dyscrasia. An overlooked caused of adult onset sensorimotor neuropathy.
复制标题
淀粉样变性伴浆细胞恶液质。
DOI：
10.1001/archneur.1977.00500160023003
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发表时间：
1977
影响因子：
--
通讯作者：
Ignaczak Tf
中科院分区：
文献类型：
--
作者：
J. Trotter;W. Engel;Ignaczak Tf
在10名以前未确诊的患者中，我们发现过去的“原发”非遗传性淀粉样变性是中老年患者主要发生的以感觉、疼痛和过度感觉为主的远端神经病的一个被忽视的原因。这些症状与直立性低血压、腹泻或便秘、心脏异常和男性阳萎有关(在没有糖尿病的情况下)。组织诊断是通过肌肉活检标本新鲜冰冻切片中淀粉样蛋白的结晶紫色异染色症快速做出的。免疫球蛋白和骨髓证据表明，10名患者中有8名出现浆细胞异型，表明这种类型的淀粉样变性的神经病变实际上继发于浆细胞性异常蛋白血症。用马法兰和泼尼松治疗无效。
In ten previously undiagnosed patients, we have found erstwhile-"primary" nonhereditary amyloidosis as an overlooked cause of a predominately sensory, painful, and hyperesthetic distal neuropathy occurring in middle-age and older patients. These symptoms, associated with orthostatic hypotension, diarrhea or constipation, cardiac abnormality, and male impotence are virtually diagnostic (in the absence of diabetes mellitus). Tissue diagnosis is quickly made by crystal-violet metachromasia of amyloid in fresh-frozen sections of a muscle biopsy specimen. Immunoglobulin and bone marrow evidence of plasma cell dyscrasia in eight of the ten patients suggests that the neuropathy in this form of amyloidosis is actually secondary to a plasma-cell-originating dysproteinemia. Therapy with melphalan and prednisone was not of benefit.