Myelodysplastic syndrome presenting as a Behçet's-like disease with aortitis

BMJ Case Rep. 2018 Mar 15:2018:bcr2017220649. doi: 10.1136/bcr-2017-220649.

Abstract

A 46-year-old Hispanic man presented with fever, genital ulcers, left eye redness and chest pain. Physical examination was notable for a healed oral ulcer and scrotal ulcers, and bilateral superficial thrombophlebitis. He was found to have new-onset pancytopenia. CT of the chest showed pericardial and pleural effusions and rapidly progressing inflammation of the aortic arch and ascending vessels. Although the patient had Behcet's disease (BD)-like symptoms, pancytopenia could not be explained by the diagnosis, prompting a bone marrow biopsy which showed myelodysplastic syndrome. This report highlights the importance of excluding alternate disorders before making a diagnosis of Behcet's disease if atypical, BD-incompatible or incomplete constellations of symptoms and findings are present.

Keywords: haematology (incl blood transfusion); rheumatology; vasculitis.

Publication types

  • Case Reports

MeSH terms

  • Aortitis / diagnosis
  • Behcet Syndrome / diagnosis
  • Bone Marrow Examination
  • Diagnosis, Differential
  • Fever / etiology
  • Humans
  • Male
  • Middle Aged
  • Myelodysplastic Syndromes / complications
  • Myelodysplastic Syndromes / diagnosis*
  • Myelodysplastic Syndromes / diagnostic imaging
  • Myelodysplastic Syndromes / pathology
  • Tomography, X-Ray Computed