Kanoh T, Suzuki K, Kawaguchi S
Department of Hematology and Oncology, Graduate School of Medicine, Kyoto University.
Rinsho Ketsueki. 1998 Dec;39(12):1157-62.
Primary Sjögren's syndrome (primary SS) is characterized by lymphocytic and plasma cell infiltration of the lacrimal and salivary glands, sometimes extending to extraglandular sites. An increased incidence of B-cell lymphoproliferative disorders has been observed in patients with primary SS. We recently studied an unusual case of primary SS associated with multifocal nodular AL amyloidosis (amyloidomas), located in the lower respiratory tract and oral mucosa. A 66-year-old woman with primary Sjögren's syndrome since 1966 was hospitalized in 1996 because of multifocal nodular shadows on a chest radiograph. An open lung biopsy specimen was obtained, revealing a large mass of amorphous eosinophilic material with green birefringence when stained with Congo red. Numerous plasma cells surrounded the amyloid deposits, aggregated in nodules and around blood vessels. The cytoplasm of these cells stained monotypically with anti-lambda light chain, using a peroxidase technique. Amyloid material did not stain with antibodies directed against kappa and lambda light chains, or against IgG, IgA and IgM heavy chains. Immunofixation of the serum and concentrated urine did not demonstrate monoclonal immunoglobulins. AL-amyloidomas might be a manifestation of local immunoglobulin production and amyloid formation within extramedullary plasmacytoma. This plasmacytoma might be burned out, namely, overcome by the deposits and no longer recognizable.
原发性干燥综合征（原发性SS）的特征是泪腺和唾液腺出现淋巴细胞和浆细胞浸润，有时可延伸至腺外部位。在原发性SS患者中观察到B细胞淋巴增殖性疾病的发病率增加。我们最近研究了一例罕见的原发性SS病例，该病例与多灶性结节性AL淀粉样变性（淀粉样瘤）相关，病变位于下呼吸道和口腔黏膜。一名自1966年起患有原发性干燥综合征的66岁女性于1996年因胸部X线片上出现多灶性结节阴影而住院。获取了一份开胸肺活检标本，结果显示有大量无定形嗜酸性物质，用刚果红染色后呈绿色双折射。大量浆细胞围绕着淀粉样沉积物，聚集成结节并围绕血管分布。使用过氧化物酶技术，这些细胞的细胞质用抗λ轻链单克隆染色。淀粉样物质不与针对κ和λ轻链、或针对IgG、IgA和IgM重链的抗体发生染色反应。血清和浓缩尿液的免疫固定电泳未显示单克隆免疫球蛋白。AL淀粉样瘤可能是髓外浆细胞瘤内局部免疫球蛋白产生和淀粉样蛋白形成的一种表现。这种浆细胞瘤可能已消退，即被沉积物所取代，不再能够识别。
