Purpose

Lymphoproliferative disease in common variable immunodeficiency disease (CVID) is heterogeneous in pathogenesis and ranges from non-malignant lymphoid hyperplasia to lymphoma.

Methods

The United States Immunodeficiency Network (USIDNET) patient registry was queried for lymphoproliferative diseases reported in CVID patients. Diagnoses included as possible manifestations of lymphoproliferation included lymphadenopathy, lymphoid hyperplasia, lymphocytic inflammation, lymphocytosis, and gammopathy.

Results

Among 1091 CVID patients, lymphoproliferative conditions were reported in 17.2% (N = 188). These conditions included lymphadenopathy (N = 192, 12.3%), lymphoid hyperplasia or lymphocytic inflammation (N = 50, 4.6%), lymphocytosis (N = 3, 0.3%), and gammopathies (N = 3, 0.3%). Of the 188 patients with lymphoproliferative conditions, 15 (8%) also had a diagnosis of lymphoma, while the remaining 173 (92%) did not. Nine (4.8%) had a diagnosis of non-lymphomatous malignancy including basal cell carcinoma (N = 3, 1.6%), thyroid carcinoma (N = 2, 1.1%), gynecologic cancer (N = 2, 1.1%), testicular cancer (N = 1), and vocal cord carcinoma (N = 1). CVID patients with lymphoma were older than patients with lymphoproliferative disease who did not have a diagnosis of lymphoma at the time of analysis (median age 49 vs. 35 years, p = 0.005). CVID patients with lymphoproliferative disease had 2.5 times higher odds of having chronic lung disease compared with those with lymphoma (OR = 0.4, p = 0.049). There were no significant differences in the frequency of autoimmune, gastrointestinal, hepatic, or granulomatous disease between these populations.

Conclusions

While CVID patients are at increased risk for lymphoma, lymphoproliferation may be observed in the absence of a concurrent hematologic or solid tumor malignancy.

中文翻译：

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CVID 中的淋巴增生性疾病：来自美国患者登记处的类型和频率报告。

目的

常见变异免疫缺陷病 (CVID) 中的淋巴组织增生性疾病的发病机制具有异质性，范围从非恶性淋巴样增生到淋巴瘤。

方法

对美国免疫缺陷网络 (USIDNET) 患者登记处查询了 CVID 患者中报告的淋巴组织增生性疾病。诊断包括淋巴增生的可能表现，包括淋巴结病、淋巴样增生、淋巴细胞炎症、淋巴细胞增多症和丙种球蛋白病。

结果

在 1091 名 CVID 患者中，17.2% ( N  = 188)报告了淋巴组织增生性疾病。这些病症包括淋巴结病 ( N  = 192, 12.3%)、淋巴组织增生或淋巴细胞炎症 ( N  = 50, 4.6%)、淋巴细胞增多症 ( N  = 3, 0.3%) 和丙种球蛋白病 ( N  = 3, 0.3%)。在 188 名患有淋巴增生性疾病的患者中，15 名 (8%) 也被诊断为淋巴瘤，而其余 173 名 (92%) 没有。9 人 (4.8%) 被诊断为非淋巴瘤性恶性肿瘤，包括基底细胞癌 ( N  = 3, 1.6%)、甲状腺癌 ( N  = 2, 1.1%)、妇科癌症 ( N  = 2, 1.1%)、睾丸癌( N = 1) 和声带癌 ( N  = 1)。CVID 淋巴瘤患者比分析时未诊断为淋巴瘤的淋巴增殖性疾病患者年龄大（中位年龄 49 岁 vs. 35 岁，p  = 0.005）。与淋巴瘤患者相比，患有淋巴组织增生性疾病的 CVID 患者患慢性肺病的几率高 2.5 倍（OR = 0.4，p  = 0.049）。这些人群之间自身免疫性疾病、胃肠道疾病、肝脏疾病或肉芽肿疾病的发生频率没有显着差异。

结论

虽然 CVID 患者患淋巴瘤的风险增加，但在没有并发血液学或实体瘤恶性肿瘤的情况下可能会观察到淋巴增生。