Rosai-dorfman disease of the larynx. a case report with molecular genetic analysis for immunoglobulin and t-cell receptor genes rearrangement

ABSTRACT

Sinus histiocytosis with massive lymphadenopathy [SHML], or Rosai-Dorfman disease [RDD], is rare histiocytic disorder originally described in 1969 as a benign Clinicopathoiogic entity characterized by massive bilateral cervical lymphadenopathy and fever. The indolent clinical course of RDD suggests a reactive disorder rather than a neopiastic process. We present a 21 year- old woman with RDD of the larynx with a recurrence of the disease after 13 months. To determine whether the lymphoplasmacytic components in these lesions clonal or polyclonal we performed a molecular testing using PCR and southern blot analyses to examine Immunoglobulin [JH] and T-cell receptor genes [TCR] genes rearrangement. The analysis showed polyclonal pattern [germline] for JH and TCR. Surface marker analysis by flow cytometry and immunohistochernistry revealed a mixed population of T and B cells with no evidence of light or heavy chain restriction. This indicates that the lymphoplasmacytic proliferation in RDD is reactive and consistent with the indolent behavior