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Behçet's disease associated with chronic myelogenous leukemia and chronic graft-vs-host disease

Behçet’s disease (BD) is an idiopathic, chronic and recurrent multi-system disease [1,2]. Although the cause and the pathogenesis of the disease remains unknown, genetic, immunologic and infectious factors are suspected [3]. Chronic myelogenous leukemia (CML) is a myeloproliferative disorder originating from abnormal stem cells [4]. The association of BD and CML is very rare. In some cases this association occured in patients receiving interferon-a therapy [5 – 7]. In this report, we present a man with CML and chronic graft-vs-host disease (GVHD) who developed characteristic features of BD. A 29-year-old man was admitted to our clinic with recurrent aphthous stomatitis (more than three times a year) since 1999, genital and extra-genital ulcerations, erythema nodosum-like lesions and papulopustular lesions on the extremities since 2002. He also had a history of recurrent oral moniliasis since 1998. On physical examination there were multiple aphthous ulcers on the oral mucosa, white plaques on the tongue, 2 – 3 mm sized erythematous papulopustular lesions over the extremities, a 2 – 3 cm sized ulceration over the sacral area and erythema nodosum-like lesions on the lower extremities. There were also brown-atrophic patches on his face, neck, presternal area and back (Figure 1). On KOH examination the patient was diagnosed to have moniliasis on his tongue. Ophthalmologic examination revealed dryness of the eyes. The examination of the joints was normal and no neurological abnormalities were detected. Hematological and biochemical tests were within normal limits and the patient was shown to have HLA-A2, -A23, -B49, -B52, -BW4, -BW6, -CW4, -CW7 phenotype. The skin pathergy test was positive using the standardized method [8]. The patient was diagnosed with BD according to the International Study Group criteria. The patient had also been diagnosed with chronic myeloblastic leukemia in 1991. He had a splenectomy in 1992 and an allogenous bone marrow transplantation in 1993. He received leucocyte infusion in June 1998 and interferon-a therapy in June and July 1998. In August 1998 he developed generalized erythema all over his body. The patient was diagnosed with acute GVHD and was treated with cyclosporine and prednisone. Four-months later he developed brown-athrophic patches especially on his face, upper extremities and trunk and was diagnosed to have chronic GVHD. Three-months later he developed aphthous stomatitis and then other symptoms of BD in 2002. BD is a chronic, multi-system disorder, presenting with exacarbations and remissions. The disease is characterized by vasculitis of arteries and veins of any size and may produce a wide variety of symptoms. BD has one cardinal feature; recurrent aphthous stomatitis and at least two of the following: recurrent genital ulceration, eye lesions, skin lesions or a positive pathergy test. Skin lesions include erythema nodosum-like lesions, papulopustular or acneiform nodules and pseudofolliculitis. Bilateral, non-granulomatous panuveitis is generally observed as eye involvement. Other manifestations are arthritis, neurological disorders, vascular, pulmonary, cardiac and renal involvement [1,2]. The association of CML and BD is very rare; only eight