Concurrent diagnosis of Sjogren’s syndrome and polycthemia vera: a rare co-existence

Serkan Ünal, S. Kocaer
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Abstract

Primary Sjogren's Syndrome(pSS) is a chronic autoimmune disease and focal lymphocytic infiltration of the exocrine glands is the key pathological feature (1). Polycythemia vera(PV) is a chronic myeloproliferative disorder characterized by JAK-2 mutations and increased red blood cell mass(2,3). Autoimmune diseases and hematological malignancies can often be observed together. Patients with SS are at a higher risk of developing lymphoproliferative diseases mainly low-grade B-cell lymphomas and predominantly marginal zone lymphoma. Moreover, PV can rarely be presented with paraneoplastic events including small vessel vasculitis (2). However, PV and pSS co-existence had never been reported. We here want to present a case of concurrently diagnosed PV and pSS.
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同时诊断出 Sjogren's 综合征和多血症:罕见的并存现象
原发性斯约格伦综合征(pSS)是一种慢性自身免疫性疾病,外分泌腺局灶性淋巴细胞浸润是其主要病理特征(1)。多发性红细胞症(PV)是一种慢性骨髓增生性疾病,其特征是 JAK-2 基因突变和红细胞增多(2,3)。自身免疫性疾病和血液恶性肿瘤常常同时出现。SS 患者罹患淋巴增生性疾病的风险较高,主要是低级别 B 细胞淋巴瘤和边缘区淋巴瘤。此外,PV 在极少数情况下会出现副肿瘤事件,包括小血管炎(2)。然而,PV 和 pSS 同时存在的病例从未报道过。我们在此介绍一例同时确诊的 PV 和 pSS 病例。
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