Behcet's Disease and T-Cell Large Granular Lymphocytic Leukemia: Two Case Reports and a Hypothesis on a Common Pathogenesis


ATAŞ Ü., Tazegul G., YÜCEL O. K., SALİM O., YAZISIZ V., ÜNDAR L.

TURKISH JOURNAL OF IMMUNOLOGY, cilt.8, sa.2, ss.94-99, 2020 (ESCI) identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 8 Sayı: 2
  • Basım Tarihi: 2020
  • Doi Numarası: 10.25002/tji.2020.1284
  • Dergi Adı: TURKISH JOURNAL OF IMMUNOLOGY
  • Derginin Tarandığı İndeksler: Emerging Sources Citation Index (ESCI), Scopus, Academic Search Premier, EMBASE, TR DİZİN (ULAKBİM)
  • Sayfa Sayıları: ss.94-99
  • Anahtar Kelimeler: Behcet's disease, autoimmunity, STAT3, T-Cell large granular lymphocytic leukemia, TNFAIP3, SERUM INTERLEUKIN-18, ACTIVATION, STAT3, IL-18, LGL, EXPRESSION
  • Akdeniz Üniversitesi Adresli: Evet

Özet

T-cell large granular lymphocytic leukemia (T-LGL) is a rare disorder, characterized by a chronic course, autoimmune manifestations and autoantibodies, cytopenias and circulating cytotoxic T-lymphocytes. T-LGL leukemia usually manifests with hematological involvement and co-existing autoimmune and/or autoinflammatory conditions. Behcet's disease (BD) is a chronic inflammatory disorder with recurrent oral and genital ulcers, uveitis, other systemic findings such as neurologic involvement, vasculitis and arthritis. Pathogenesis of BD is still poorly understood. However, a polarization of the Th1/Th2 immune response toward the Th1 pathway, and Th17 involvement have been shown. Herein, we present two cases of T-LGL co-existing with BD, second and third cases in the literature. We review, discuss and hypothesize a possible pathogenetic association between BD and T-LGL.