Background

The association of nonparaneoplastic pemphigus with comorbid hematologic malignancies has yet to be established.

Objective

To estimate the association between pemphigus and the common types of hematologic malignancies.

Methods

A cross-sectional study was conducted comparing pemphigus patients with age-, sex- and ethnicity-matched control subjects regarding the prevalence of 6 comorbid hematologic malignancies. The study was performed using the computerized database of Clalit Health Services ensuring the availability of 4.5 million patients.

Results

The study included 1985 pemphigus patients and 9874 control subjects. The prevalence of chronic leukemia (0.9% vs 0.4%, odds ratio [OR] 2.1, 95% confidence interval [CI] 1.2-3.6), multiple myeloma (0.8% vs 0.4%, OR 2.2, 95% CI 1.2-3.9), and non-Hodgkin lymphoma (1.8% vs 1.2%, OR 1.5, 95% CI 1.0-2.2) was greater in patients with pemphigus than in controls. The association with chronic leukemia remained significant following the adjustment for immunosuppressive therapy (adjusted OR 2.0, 95% CI 1.1-3.7). No significant associations were observed between pemphigus and acute leukemia, Hodgkin lymphoma, myelodysplastic syndrome, and polycythemia vera.

Limitations

Lack of immunopathologic validation of the diagnosis of pemphigus.

Conclusion

A significant association was observed between pemphigus and chronic leukemia, multiple myeloma, and non-Hodgkin lymphoma. Further research is warranted to establish this observation in other cohorts.

中文翻译：

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天疱疮和血液系统恶性肿瘤：一项基于人群的11859名患者的研究

背景

非副肿瘤性天疱疮与合并性血液恶性肿瘤的关联尚未建立。

客观的

估计天疱疮与血液系统恶性肿瘤的常见类型之间的关联。

方法

进行了一项横断面研究，比较了天疱疮患者与年龄，性别和种族相匹配的对照对象在6种合并血液恶性肿瘤中的患病率。该研究是使用Clalit Health Services的计算机数据库进行的，可确保450万患者的可用性。

结果

该研究纳入了1985名天疱疮患者和9874名对照对象。慢性白血病的患病率（0.9％vs 0.4％，优势比[OR] 2.1，95％置信区间[CI] 1.2-3.6），多发性骨髓瘤（0.8％vs 0.4％，OR 2.2，95％CI 1.2-3.9） ，天疱疮患者的非霍奇金淋巴瘤（1.8％vs 1.2％，OR 1.5，95％CI 1.0-2.2）比对照组大。调整免疫抑制疗法后，与慢性白血病的相关性仍然很显着（调整为OR 2.0，95％CI 1.1-3.7）。天疱疮与急性白血病，霍奇金淋巴瘤，骨髓增生异常综合症和真性红细胞增多症之间未发现显着相关性。

局限性

缺乏天疱疮诊断的免疫病理学验证。

结论

天疱疮与慢性白血病，多发性骨髓瘤和非霍奇金淋巴瘤之间存在显着相关性。有必要进行进一步的研究以在其他队列中建立这种观察。