与干燥综合征或 HCV 感染相关的冷球蛋白血症：基于骨髓受累模式、淋巴瘤演变以及腮腺切除术后实验室检查的差异。

Cryoglobulinaemia related to Sjogren's syndrome or HCV infection: differences based on the pattern of bone marrow involvement, lymphoma evolution and laboratory tests after parotidectomy.

机构信息

Rheumatology Clinic, Department of Medical and Biological Sciences, University Hospital 'S. Maria della Misericordia', Piazzale Santa Maria Misericordia 1, 33100 Udine, Italy.

出版信息

Rheumatology (Oxford). 2012 Apr;51(4):627-33. doi: 10.1093/rheumatology/ker407. Epub 2011 Dec 30.

DOI:10.1093/rheumatology/ker407

PMID:22210656

Abstract

OBJECTIVE

The relationship of cryoglobulinaemia with lymphoproliferation of mucosa-associated lymphoid tissue (MALT) as risk factors for lymphoma evolution in SS remains to be clarified. The different biologic background of SS-related cryoglobulinaemia as compared with cryoglobulinaemia linked to HCV infection was clarified by different clinical and biologic approaches.

METHODS

B-cell clonal expansion was analysed in the bone marrow of 27 consecutive cases with primary SS and mixed cryoglobulinaemia, HCV unrelated, in comparison with 55 HCV-related patients with cryoglobulinaemic vasculitis (CV) without SS. The results were related to the possible occurrence and localization of B-cell lymphoma in the single case. Secondly, the prevalence of mixed cryoglobulinaemia was investigated in 41 unselected patients with primary SS showing either parotid myoepithelial sialadenitis (MESA) or a frank B-cell non-Hodgkin's lymphoma. Thirdly, the levels of serum cryoglobulins and RF were followed in one patient with primary SS, CV and parotid B-cell lymphoma of MALT after bilateral subtotal parotidectomy.

RESULTS

A polyclonal pattern of B expansion in the bone marrow was significantly more frequent in SS-related (19/27 cases) than in HCV-related cryoglobulinaemia (19/55) (P = 0.003). Cryoglobulins were positive in a fraction of patients with SS and malignant lymphoma or with parotid MESA (13/18 and 7/23, respectively), whereas MALT involvement by the lymphoproliferative disorder was the rule. Finally, the levels of serum cryoglobulins and RF markedly decreased in the SS patient undergoing bilateral subtotal parotidectomy.

CONCLUSION

Lymphoproliferation of MALT appears as the biologic background of cryoglobulinaemia in SS, differently from HCV-related cryoglobulinaemia.

摘要

目的

在 SS 中，冷球蛋白血症与黏膜相关淋巴组织（MALT）的淋巴增生之间的关系作为淋巴瘤演变的风险因素仍需阐明。通过不同的临床和生物学方法，阐明了 SS 相关冷球蛋白血症与 HCV 感染相关冷球蛋白血症的不同生物学背景。

方法

分析了 27 例原发性 SS 合并混合性冷球蛋白血症（HCV 无关）和 55 例 HCV 相关伴有冷球蛋白血症性血管炎（CV）但无 SS 的患者的骨髓中 B 细胞克隆扩增。将结果与单个病例中 B 细胞淋巴瘤的可能发生和定位相关联。其次，在 41 例未选择的原发性 SS 患者中调查了混合性冷球蛋白血症的患病率，这些患者表现为腮腺肌上皮涎腺炎（MESA）或明显的 B 细胞非霍奇金淋巴瘤。第三，在一例原发性 SS、CV 和腮腺 MALT B 细胞淋巴瘤患者中，在双侧腮腺大部切除术后，随访血清冷球蛋白和 RF 水平。

结果

骨髓中 B 细胞扩增的多克隆模式在 SS 相关（19/27 例）明显比 HCV 相关冷球蛋白血症（19/55 例）更常见（P=0.003）。冷球蛋白在一部分 SS 伴恶性淋巴瘤或腮腺 MESA 的患者中呈阳性（分别为 13/18 和 7/23），而 MALT 受累是淋巴增生性疾病的常见模式。最后，在接受双侧腮腺大部切除术的 SS 患者中，血清冷球蛋白和 RF 的水平明显降低。