Clonal rearrangement for immunoglobulin and T-cell receptor genes in systemic Castleman's disease. Association with Epstein-Barr virus

Am J Pathol. 1988 Apr;131(1):84-91.


Castleman's disease is a morphologically and clinically heterogeneous lymphoproliferative disorder. Both a localized benign variant and an aggressive form with systemic manifestations have been described. To investigate the differences between these variants of Castleman's disease, the authors analyzed lymph node DNA from 4 patients with the localized type and 4 with the systemic type of Castleman's disease for immunoglobulin and T-cell receptor gene rearrangements. The role of Epstein-Barr virus (EBV) and cytomegalovirus (CMV) was also studied by viral genomic DNA probes. They detected clonal rearrangements in 3 of the 4 patients with the systemic variant of Castleman's; no patients with localized disease had rearrangements. Copies of EBV genome were also detected in 2 of the 3 patients with clonal rearrangements. These results suggest that systemic Castleman's disease is a disorder distinct from the classical localized variant in that it may evolve into a clonal lymphoproliferation.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Castleman Disease / genetics
  • Castleman Disease / immunology*
  • Castleman Disease / microbiology
  • Castleman Disease / pathology
  • Genes*
  • Genes, Immunoglobulin*
  • Genes, Viral*
  • Herpesvirus 4, Human / genetics*
  • Herpesvirus 4, Human / isolation & purification
  • Humans
  • Immunoglobulins / genetics*
  • Lymph Nodes / immunology*
  • Lymph Nodes / microbiology
  • Lymph Nodes / pathology
  • Middle Aged
  • Receptors, Antigen, T-Cell / genetics*


  • Immunoglobulins
  • Receptors, Antigen, T-Cell