| Literature DB >> 11531802 |
S A Grabczynska1, C T Toh, N Francis, C Costello, C B Bunker.
Abstract
We report a patient with sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) who presented with widespread nodal and extranodal involvement affecting the skin, orbits and nasal sinuses, complicated by the development of autoimmune haemolytic anaemia. The aetiology and pathogenesis of this multisystem disorder are unknown but are thought to represent a reactive histiocytic process to an infective agent rather than a neoplastic or other primary condition. Prognosis is generally good but clinical or laboratory evidence of immune dysfunction tends to predict a poorer outcome. We describe the clinical course of the patient and review the literature on this disease.Entities:
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Year: 2001 PMID: 11531802 DOI: 10.1046/j.1365-2133.2001.04325.x
Source DB: PubMed Journal: Br J Dermatol ISSN: 0007-0963 Impact factor: 9.302