Hachulla E, Janin A, Masson H, Lepoutre F, Smadja D, Bataille D, Hatron P Y, Gosselin B, Devulder B
Service de Médecine Interne, Hôpital Huriez, Lille.
Rev Med Interne. 1990 Jul-Aug;11(4):329-32. doi: 10.1016/s0248-8663(05)80869-0.
There are few reports of amyloidosis diagnosed by deliberate biopsy of accessory salivary glands. Usually, a biopsy performed for dry mouth syndrome reveals an unsuspected amyloidosis. We report the case of 2 patients with lambda-type light chain monoclonal gammapathy complicated by generalized amyloidosis and in whom biopsy of the accessory salivary glands showed signs of amyloidosis. In the first patient accessory salivary gland biopsy was performed because these glands were enlarged, and the monoclonal dysglobulinaemia was subsequently diagnosed by serum immunoelectrophoresis. In the second patient with nephrotic syndrome, renal biopsy could not be carried out owing to the presence of a renal malformation; amyloidosis was confirmed by periumbilical fat aspiration, and a systematic biopsy of accessory salivary glands also showed evidence of amyloidosis. Biopsy of accessory salivary glands seems to be a particularly simple and safe method to detect generalized amyloidosis in patients with chronic inflammatory disease or monoclonal dysglobulinaemia.
关于通过对副唾液腺进行特意活检来诊断淀粉样变性的报道很少。通常,因口干综合征进行的活检会意外发现淀粉样变性。我们报告了2例λ型轻链单克隆丙种球蛋白病并发全身性淀粉样变性的病例,其副唾液腺活检显示有淀粉样变性迹象。首例患者因副唾液腺肿大而进行活检,随后通过血清免疫电泳诊断出单克隆球蛋白血症。第二例患有肾病综合征的患者,由于存在肾脏畸形而无法进行肾活检;通过脐周脂肪抽吸确诊为淀粉样变性,对副唾液腺进行的系统活检也显示有淀粉样变性证据。对于患有慢性炎症性疾病或单克隆球蛋白血症的患者,副唾液腺活检似乎是一种特别简单且安全的检测全身性淀粉样变性的方法。