Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case with widespread nodal and extra nodal dissemination.

A fatal case of sinus histiocytosis with massive lymphadenopathy (SHML) is described in a male patient who presented at 11 years-of-age with swelling of the malar region and nasal obstruction due to the disease and who died aged 28 in uraemic coma following renal involvement. At autopsy SHML tissue extended into the hilum of both lungs and from the retroperitoneal tissue into the pancreas and kidneys. The appearances suggest that the extension of the disease into these organs may have been due to retrograde lymphatic spread from involved lymph nodes. Replacement of the atypical sinus histiocyte by fibrous tissue was seen in the cervical lymph nodes and this may be the usual mode of resolution of the lesion. The nature of SHML is discussed in relation to a possible infectious aetiology and defects in the host immune system. Finally, it is noted that the term 'massive lymphadenopathy' is not applicable to all cases and that sinus histiocytosis in not appropriate for the now frequently recognized extranodal manifestations of the disease. It is proposed that the term 'lymphophagocytic histiocytosis syndrome' should be adopted or that the eponymous title 'Rosai-Dorfman disease' should be used.