J Rheum Dis.  2012 Feb;19(1):55-58. 10.4078/jrd.2012.19.1.55.

A Case of Behcet's Disease Associated with Myelofibrosis

• Kim JW
• Lee SH
• Song R
• Lee YA
• Hong SJ
• Yang HI

• KMID: 2223122
• DOI: http://doi.org/10.4078/jrd.2012.19.1.55

Abstract

Behcet'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.

Keyword

Behcet's disease; Myelofibrosis; Hematologic involvement

MeSH Terms

Anemia
Bone Marrow
Bone Marrow Examination
Hemoglobins
Humans
Leukemia
Middle Aged
Primary Myelofibrosis
Splenomegaly
Systemic Vasculitis
Hemoglobins

Figure

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  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).

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  Figure 2 Bone marrow biopsy sections showing coarse bundles of collagen fibers (Masson's trichrome stain). (A) ×200, (B) ×1,000.

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  Figure 3 Reticulin staining of the bone marrow showing marked diffuse reticulin fibrosis (reticulin stain, ×200).

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