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干燥综合征的一种罕见病因。

An unusual cause for sicca syndrome.

作者信息

Kulkarni U P, Gokhale Y A, Raut P M, Dargad R R

机构信息

Department of Medicine, Lokmanya Tilak Municipal Medical College and General Hospital, Sion, Mumbai, India.

出版信息

J Postgrad Med. 2011 Apr-Jun;57(2):129-30. doi: 10.4103/0022-3859.81872.

Abstract

We present a case of a 47-year-old female who presented with sicca symptoms since three months. As per the Revised International Classification Criteria for Sjögren's syndrome, patient was diagnosed as primary Sjögren's syndrome (SS). Patients with SS are known to have circulating monoclonal immunoglobulins. Serum electrophoresis revealed M band with serum gamma globulin concentration of 46 g/L. Bone marrow aspiration revealed 28% plasma cells. In absence of myeloma-related organ damage, a diagnosis of smouldering myeloma (MM) was made. Patient was treated with thalidomide and dexamethasone. Sicca symptoms resolved with anti-myeloma treatment. Although MM can occur as a complication of SS, MM can also rarely present as SS. In the present case, the short duration of sicca symptoms and response of these symptoms to anti-myeloma treatment support the diagnosis of MM presenting as SS. The present case highlights the importance of serum electrophoresis in patients presenting as SS.

摘要

我们报告一例47岁女性,她三个月来一直出现干燥症状。根据修订后的干燥综合征国际分类标准,该患者被诊断为原发性干燥综合征(SS)。已知SS患者存在循环单克隆免疫球蛋白。血清电泳显示M带,血清γ球蛋白浓度为46 g/L。骨髓穿刺显示浆细胞占28%。在没有骨髓瘤相关器官损害的情况下,诊断为冒烟型骨髓瘤(MM)。患者接受了沙利度胺和地塞米松治疗。干燥症状通过抗骨髓瘤治疗得以缓解。虽然MM可作为SS的并发症出现,但MM也很少表现为SS。在本病例中,干燥症状持续时间短且这些症状对抗骨髓瘤治疗有反应,支持了MM表现为SS的诊断。本病例突出了血清电泳在以SS表现的患者中的重要性。

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