Massachusetts General Hospital, 55 Fruit St, Bulfinch 165, Boston, MA, 02114, USA.
Ann and Robert H Lurie Children's Hospital of Chicago, Chicago, IL, USA.
J Clin Immunol. 2020 Apr;40(3):524-530. doi: 10.1007/s10875-020-00769-8. Epub 2020 Mar 17.
Lymphoproliferative disease in common variable immunodeficiency disease (CVID) is heterogeneous in pathogenesis and ranges from non-malignant lymphoid hyperplasia to lymphoma.
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.
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.
While CVID patients are at increased risk for lymphoma, lymphoproliferation may be observed in the absence of a concurrent hematologic or solid tumor malignancy.
普通变异性免疫缺陷病（CVID）中的淋巴增生性疾病在发病机制上具有异质性，范围从非恶性淋巴组织增生到淋巴瘤。
美国免疫缺陷网络（USIDNET）患者登记处查询了 CVID 患者报告的淋巴增生性疾病。诊断包括作为淋巴增生表现的可能形式，包括淋巴结病、淋巴组织增生、淋巴细胞炎症、淋巴细胞增多和单克隆丙种球蛋白病。
在 1091 例 CVID 患者中，有 17.2%（N=188）报告了淋巴增殖性疾病。这些疾病包括淋巴结病（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（N=3，0.3%）和单克隆丙种球蛋白病（N=3，0.3%）。在有淋巴增生性疾病的 188 例患者中，有 15 例（8%）还诊断为淋巴瘤，而其余 173 例（92%）未诊断为淋巴瘤。有 9 例（4.8%）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）。在分析时患有淋巴瘤的 CVID 患者比患有非淋巴瘤性淋巴增生性疾病的患者年龄更大（中位数年龄 49 岁 vs. 35 岁，p=0.005）。与患有淋巴瘤的患者相比，患有淋巴增生性疾病的 CVID 患者患慢性肺部疾病的几率高 2.5 倍（OR=0.4，p=0.049）。这些人群之间在自身免疫性疾病、胃肠道疾病、肝脏疾病或肉芽肿性疾病的频率方面没有显著差异。
虽然 CVID 患者患淋巴瘤的风险增加，但在没有同时发生血液系统或实体肿瘤恶性肿瘤的情况下，也可能观察到淋巴增生。
