Behcet's disease associated with malignancy: a report of 41 Chinese cases

Abstract

Objective: To identify the clinical characteristics of Behcet's disease (BD) associated with malignancies.

Method: Among 651 BD patients sequentially hospitalized in Peking Union Medical College Hospital from 1995 to 2012, 41 patients developed malignancies. Clinical characteristics were compared between patients with and without malignancies, as well as between patients with hematological and solid malignancies.

Results: The numbers of patients with various types of malignancies were listed as follows: (i) 29 cases developed hematologic malignancies which included myelodysplastic syndrome (MDS) as the most common diagnosis in 20 cases, leukemia in seven cases, anaplastic anemia in two cases and lymphoma in one case; (ii) 13 cases developed malignant solid neoplasms which included colorectal cancer in three cases, urothelial carcinoma of bladder in two cases, esophageal cancer, gastric cancer, pancreatic cancer, thyroid cancer, breast cancer, cervical cancer, renal cell carcinoma and metastatic adenocarcinoma of unknown primary in one case each. Female gender, older age and gastrointestinal tract involvement were more frequently observed among patients with malignancy than those without. Older age, longer disease duration were more commonly seen in BD patients with solid neoplasms than in those with hematologic malignancies. Exacerbation of BD manifestations during the emergence of malignancy was more frequently observed among patients with hematologic malignancies than among those with malignant solid neoplasms.

Conclusion: This study demonstrated that MDS and colorectal cancer were the most common hematologic malignancy and malignant solid neoplasm associated with BD, respectively. Gastrointestinal involvement is likely to be a risk factor for BD patients to develop malignancy. Further studies will be required to ascertain the pathogenic link between these two entities.

Keywords: Behcet' s disease; hematologic malignancy; malignant solid neoplasm.