Hematologic Manifestations and Predictors of Lymphoma Development in Primary Sjogren Syndrome Clinical and Pathophysiologic Aspects
Hematologic Manifestations and Predictors of Lymphoma Development in Primary Sjogren Syndrome Clinical and Pathophysiologic Aspects
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DOI：
10.1097/md.0b013e3181b76ab5
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发表时间：
2009-09-01
期刊：
影响因子：
1.6
通讯作者：
Moutsopoulos, Haralampos M.
中科院分区：
文献类型：
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作者：
Baimpa, Evangelia;Dahabreh, Issa J.;Moutsopoulos, Haralampos M.
原发性干燥综合征（pSS）的多种血液学表现尚未得到系统研究，其预后相关性仍不清楚。我们对536例连续患者进行了回顾性研究，以评估血液学异常的患病率及其与pSS各种疾病表现的相关性。我们的目的还在于确定非霍奇金淋巴瘤（NHL）发展的危险因素，包括总体和亚型。慢性病贫血和高丙种球蛋白血症是最常见的血液学表现，在诊断和病程中遇到的pSS。血细胞减少和腺体表现之间的单变量分析显示，淋巴细胞减少和腮腺肿大（p = 0.002），以及中性粒细胞减少和口干（p = 0.019）之间的统计学显著相关性。贫血、淋巴细胞减少、血小板减少、高丙种球蛋白血症、单克隆血清蛋白和冷球蛋白血症与腺外症状如可触及紫癜、淋巴结病和脾肿大显著相关，7.5%（95%可信区间[CI]，5.4%-10%）的患者被诊断为淋巴瘤。边缘区B细胞淋巴瘤（MZBCL）是主要的组织学类型（65%; 95%，CI，48.3%-79.4%），而弥漫性大B细胞淋巴瘤（DLBCL）占所有病例的17.5%（95% CI，7.3%-32.8%）。pSS患者NHL的发展可通过诊断时存在的简单临床和实验室因素预测：中性粒细胞减少（p = 0.041）、冷球蛋白血症（p = 0.008）、脾肿大（p = 0.006）、淋巴结病（p = 0.021）和低C4水平（p = 0.009）。携带任何这些因素的患者患NHL的风险比没有任何风险因素的患者高5倍以上。上述疾病特征可预测MZBCL的后续发展;诊断时淋巴细胞减少症（p = 0.044）的存在可作为非MZBCL（最常见为DLBCL）发展的风险因素。慢性疾病贫血和高丙种球蛋白血症是pSS诊断时和病程中常见的血液学表现。诊断时的中性粒细胞增多症和冷球蛋白血症与淋巴瘤发展的所有风险增加显著相关。
The diverse hematologic manifestations of primary Sjogren (pSS) have not been systematically investigated, and their prognostic relevance remains unclear. We conducted a retrospective study of 536 consecutive patients followed our institution to assess the prevalence of hematologic abnormalities and their associations with various disease manifestations in pSS. We also aimed to identify risk factors for the development of non-Hodgkin lymphoma (NHL) overall and by subtype.Anemia of chronic disease and hypergammaglobulinemia were the most prevalent hematologic manifestations encountered at diagnosis and during the Course of pSS. Univariate analysis between cytopenias and glandular manifestations revealed a statistically significant correlation between lymphocytopenia and parotid gland enlargement (p = 0.002), as well as between neutropenia and xerostomia (p = 0.019). Anemia, lymphocytopenia, thrombocytopenia, hypergammaglobulinemia, the presence of monoclonal serum proteins, and cryoglobulinemia correlated significantly with the presence of extraglandular symptoms Such as palpable purpura, lymphadenopathy, and splenomegaly.Lymphoma was diagnosed in 7.5% (95% confidence interval [CI], 5.4%-10%) of patients. Marginal zone B-cell lymphomas (MZBCLs) were the predominant histologic type (65%; 95%, CI, 48.3%-79.4%), while diffuse large B-cell lymphomas (DLBCLs) accounted for 17.5% (95% CI, 7.3%-32.8%) of all cases. The development of NHL in patients With pSS Could be predicted by the presence of simple clinical and laboratory factors at diagnosis: neutropenia ( p = 0.041), cryoglobulinemia (p = 0.008), splenomegaly (p = 0.006), lymphadenopathy (p = 0.021), and low C4 levels (p = 0.009). Patients carrying any of these factors had a more than 5-fold increased risk of NHL compared to patients with no risk factors at all. The above set of disease characteristics Could predict Subsequent development of MZBCL; the presence of lymphocytopenia (p = 0.044) at diagnosis served as a risk factor for the development of a non-MZBCL, most commonly DLBCL.Anemia of chronic disease and hypergammaglobulinemia are common hematologic manifestations at diagnosis and during the course of pSS. Neutropenia and cryoglobulinemia at diagnosis are significantly associated with all increased risk of lymphoma development.