Revlimid or thalidomide for Rosai Dorfman – pro
Rosai and Dorfman first described sinus histiocytosis with massive lymphadenopathy in 1969 as a benign lymphohistiocytic proliferative condition involving lymph nodes. The classic clinical presentation of Rosai-Dorfman disease is massive painless cervical lymphadenopathy, but can also include nasal obstruction, tonsillar enlargement, or hearing abnormalities. It and may be accompanied by leukocytosis, elevated erythrocyte sedimentation rate (ESR), weight loss, and hypergammaglobulinemia. While lymph node involvement often dominates the clinical presentation, other anatomic sites are involved in 30-40% of cases, most commonly the skin, upper respiratory tract, orbit and testes.The report on which the proposed use is based is the first and only so far published supporting evidence for lenalidomide in Rosaai Dorfman Disease. It is understood that this is an inadequate basis for approval.
There is reports of cases that responded to a related drug, thalidomide, but a prospective trial would be needed before routine use can be supported.
Rubinstein M et al, Lenalidomide in the treatment of Rosai Dorfman disease–a first in use report.Am J Hematol. 2016 Feb;91(2):E1.
Chen E et al, Rosai-Dorfman disease successfully treated with thalidomide. JAAD Case Rep. 2016 Sep 28;2(5):369-372. eCollection 2016.