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Behçet’s disease associated with malignancies. Report of two cases and review of the literature

V.G. Kaklamani¹, A. Tzonou², P.G. Kaklamanis³

¹Feinberg School of Medicine, Northwestern University, Chicago, USA; ²Department of Hygiene and Epidemiology, Medical School, Athens University, Greece; ³Department of Rheumatology, Athens Medical Center, Athens, Greece.

ABSTRACT
Objective
To investigate the incidence of malignancies in a cohort of Behçet’s disease patients and review the world literature.

Methods
Our database of 128 patients was searched and the age standardized rate (ASR) for cancer was calculated. Furthermore, we performed a MEDLINE search from 1970 through 2003, as well as, a search in the proceedings of international conferences for cases of malignancies associated with Behçet’s disease.

Results
Two of our 128 patients with Behçet’s disease were found to have solid tumors. One male had lung cancer and the other female had kidney cancer. The ASR for cancer cases in our population was investigated and it was found to be 1,600 per 100,000 in 10 years. The ASR for cancer cases in Greece according to WHO is 272.51 per 100,000 per year and therefore 2,725 per 100,000 in 10 years.
In the world literature 112 cases of malignancies associated with Behçet’s disease were found: Sixty five cases were of male patients and 46 of female with 1 case of unknown gender. The solid malignancies associated with Behçet’s disease included cases of bladder, breast, uterus, thyroid and stomach cancer, whereas haematological malignancies included leukemia, myelodysplastic syndrome, lymphoma, multiple myeloma, Hodgkin’s disease and lymphosarcoma. The treatment administered in these patients with their disease is also reported.

Conclusion
The age standardized rate of cancer in our population was lower than that of the general population in Greece, although the difference was not statistically significant. However, there is discrepancy in the world literature and the possibility of development of malignancies in Behçet’s disease patients should not be ignored.

Key words
Behçet’s disease, malignancies.

Please address correspondence to: Phaedon Kaklamanis, MD, 61 Ipsilantou St., Athens 11521, Greece.
E-mail: Virginia @hol.gr