Klussmann J P, Guntinas-Lichius O, Heilig B, Wagner M, Jungehülsing M, Michel O
Klinik und Poliklinik für Hals-Nasen-Ohrenheilkunde, Universität zu Köln.
HNO. 1999 Jul;47(7):637-41. doi: 10.1007/s001060050437.
Sjögren's syndrome is an important autoimmune disease in the head and neck. Patients have an increased arrival risk of up to 6% per year for developing B-cell lymphomas, including mucosa-associated lymphoid tissue (MALT) lymphomas. The following case report shows this relation and the difficulty of differentiating clinically recurrent swelling of the parotid gland in Sjögren's syndrome from malignant lymphoma. A 64-year-old woman had a 2-year history of indolent, recurrent swelling of both parotid glands. Blood examination showed elevated ESR and a hypergammaglobulinemia. Immunosuppressive therapy produced no improvement. Two years after the diagnosis of Sjögren's syndrome, swelling of the left parotid gland persisted. Superficial parotidectomy of the left side was performed and histopathological examination revealed a MALT-related lymphoma. Subsequent parotidectomy of the right side also showed infiltration of the gland by a MALT lymphoma. Postoperative radiation therapy was given. During the follow-up period no recurrence or systemic disease was detected. Patients with Sjögren's syndrome should be examined regularly by the otolaryngologist. If a lymphoma cannot be ruled out, open biopsy must be considered for histological diagnosis. Prognostic factors for developing a lymphoma are possibly a high ESR and hypergammaglobulinemia. Further prognostic factors have to be evaluated.
干燥综合征是头颈部一种重要的自身免疫性疾病。患者每年发生B细胞淋巴瘤（包括黏膜相关淋巴组织（MALT）淋巴瘤）的风险增加，高达6%。以下病例报告显示了这种关系，以及在临床上区分干燥综合征腮腺反复肿胀与恶性淋巴瘤的困难。一名64岁女性有两年双侧腮腺无痛性反复肿胀病史。血液检查显示血沉升高和高球蛋白血症。免疫抑制治疗无效。干燥综合征诊断两年后，左侧腮腺仍持续肿胀。对左侧进行了腮腺浅叶切除术，组织病理学检查显示为MALT相关淋巴瘤。随后右侧腮腺切除术也显示有MALT淋巴瘤浸润腺体。术后进行了放射治疗。在随访期间未发现复发或全身性疾病。干燥综合征患者应由耳鼻喉科医生定期检查。如果不能排除淋巴瘤，必须考虑进行开放性活检以进行组织学诊断。发生淋巴瘤的预后因素可能是血沉升高和高球蛋白血症。还必须评估其他预后因素。
