Sinus histiocytosis with massive lymphadenopathy: a new case in an adult with anti factor VIIi antibody
Cortet, P.; Chalopin, J.M.; Besancenot, J.F.; Knopf, J.F.; Michiels, R.; Lorenzini, J.L.; Guy, H.; Rifle, G.
Annales de Medecine Interne 131(6): 357-360
1980
ISSN/ISBN: 0003-410X PMID: 6781391 Document Number: 168260
The case history of a 71 year old french man with a lethal sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman's Syndrome) is reported. The histological features were typical but the clinical course was remarkable because of the predominant expression was a life threatening hemorrhagic disorder closely linked to a circulating anticoagulant (anti-factor VIII). The bringing to light in a picture of hyperimmunity of a self antibody and a high anti-EBV titer suggest on lymphocyte T deficit could be important in the development of the sinus histiocytosis.