Journal List > J Rheum Dis > v.19(1) > 1064015

Kim, Lee, Song, Lee, Hong, and Yang: A Case of Behçet's Disease Associated with Myelofibrosis

Abstract

Behçet's disease (BD) is a systemic vasculitis of unknown etiology that is rarely observed in association with leukemia and other hematologic disorders. We recently encountered a patient who presented with BD associated with myelofibrosis that was not attributable to other underlying causes. The patient was a 49-year-old man with a 3-year history of probable BD diagnosed by clinical findings; he was admitted because of anemia and splenomegaly. Bone marrow examination revealed myelofibrosis. After steroid therapy, the patient's symptoms of BD improved, and his hemoglobin level recovered. Therefore, we believe that the patient had BD with bone marrow involvement.

Figures and Tables

Figure 1
Aphthous lesions on the tongue and lip (A). Erythema nodosum lesions scattered over the legs (B). Multiple scrotal and perianal ulcers that developed at a later stage (C).
jrd-19-55-g001
Figure 2
Bone marrow biopsy sections showing coarse bundles of collagen fibers (Masson's trichrome stain). (A) ×200, (B) ×1,000.
jrd-19-55-g002
Figure 3
Reticulin staining of the bone marrow showing marked diffuse reticulin fibrosis (reticulin stain, ×200).
jrd-19-55-g003

References

1. Sakane T, Takeno M, Suzuki N, Inaba G. Behçet's disease. N Engl J Med. 1999. 341:1284–1291.
2. Ohno E, Ohtsuka E, Watanabe K, Kohno T, Takeoka K, Saburi Y, et al. Behçet's disease associated with myelodysplastic syndromes. A case report and a review of the literature. Cancer. 1997. 79:262–268.
3. Oh EJ, Yoon JS, Park YJ, Cho CS, Kim BK. Behçet's disease associated with myelodysplastic syndrome: a case report. J Korean Med Sci. 1999. 14:685–687.
4. International Study Group for Behçet's Disease. Criteria for diagnosis of Behçet's disease. Lancet. 1990. 335:1078–1080.
5. Jaber L, Milo G, Halpern GJ, Krause I, Weinberger A. Prevalence of Behçet's disease in an Arab community in Israel. Ann Rheum Dis. 2002. 61:365–366.
6. Cengiz M, Altundag MK, Zorlu AF, Güllü IH, Ozyar E, Atahan IL. Malignancy in Behçet's disease: a report of 13 cases and a review of the literature. Clin Rheumatol. 2001. 20:239–244.
7. Gül A. Behçet's disease: an update on the pathogenesis. Clin Exp Rheumatol. 2001. 19:5 Suppl 24. S6–S12.
8. Rotteveel FT, Kokkelink I, van Lier RA, Kuenen B, Meager A, Miedema F, et al. Clonal analysis of functionally distinct human CD4+ T cell subsets. J Exp Med. 1988. 168:1659–1673.
9. Arimura K, Arima N, Matsushita K, Akimoto M, Park CY, Uozumi K, et al. High incidence of morphological myelodysplasia and apoptotic bone marrow cells in Behçet's disease. J Clin Immunol. 2007. 27:145–151.
10. Yuo A, Kitagawa S, Suzuki I, Urabe A, Okabe T, Saito M, et al. Tumor necrosis factor as an activator of human granulocytes. Potentiation of the metabolisms triggered by the Ca2+-mobilizing agonists. J Immunol. 1989. 142:1678–1684.
11. Budak-Alpdoğan T, Demirçay Z, Alpdoğan O, Direskeneli H, Ergun T, Bayik M, et al. Behçet's disease in patients with chronic myelogenous leukemia: possible role of interferon-alpha treatment in the occurrence of Behçet's symptoms. Ann Hematol. 1997. 74:45–48.
12. Kuter DJ, Bain B, Mufti G, Bagg A, Hasserjian RP. Bone marrow fibrosis: pathophysiology and clinical significance of increased bone marrow stromal fibres. Br J Haematol. 2007. 139:351–362.
13. Cappio FC, Vigliani R, Novarino A, Camussi G, Campana D, Gavosto F. Idiopathic myelofibrosis: a possible role for immune-complexes in the pathogenesis of bone marrow fibrosis. Br J Haematol. 1981. 49:17–21.
14. Paquette RL, Meshkinpour A, Rosen PJ. Autoimmune myelofibrosis. A steroid-responsive cause of bone marrow fibrosis associated with systemic lupus erythematosus. Medicine (Baltimore). 1994. 73:145–152.
15. Pamuk GE, Pamuk ON, Orüm H, Demir M, Turgut B, Cakir N. Might platelet-leucocyte complexes be playing a role in major vascular involvement of Behçet's disease? A comparative study. Blood Coagul Fibrinolysis. 2010. 21:113–117.
TOOLS
Similar articles