Trisomy 8-positive Polycythemia Vera Complicated with Intestinal Behçet's-like Disease: A New Perspective for a Clinical Approach

Intern Med. 2022 Jun 1;61(11):1713-1719. doi: 10.2169/internalmedicine.8395-21. Epub 2021 Nov 6.

Abstract

Behçet's disease (BD) is a multisystem inflammatory disease of unknown origin. It rarely but occasionally occurs together with myelodysplastic syndrome and primary myelofibrosis. Trisomy 8 is one of the most common cytogenetic abnormalities in myeloid neoplasms; however, the association of BD with polycythemia vera (PV) and trisomy 8 has not been reported. A 70-year-old woman, diagnosed with PV and treated with hydroxyurea, had bloody stool due to multiple ulcers in the ileocecal region. Considering the lack of a response to treatment and other features, we suspected complication with intestinal Behçet's-like disease. Our case suggests relationships among BD, trisomy 8, and PV.

Keywords: Behçet's disease; polycythemia vera; trisomy 8.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Behcet Syndrome* / complications
  • Behcet Syndrome* / diagnosis
  • Behcet Syndrome* / genetics
  • Chromosomes, Human, Pair 8
  • Female
  • Humans
  • Myelodysplastic Syndromes* / complications
  • Myelodysplastic Syndromes* / diagnosis
  • Myelodysplastic Syndromes* / genetics
  • Polycythemia Vera* / complications
  • Polycythemia Vera* / diagnosis
  • Polycythemia Vera* / genetics
  • Trisomy / genetics

Supplementary concepts

  • Chromosome 8, trisomy