Rosai-Dorfman disease

(redirected from adenomegalic pseudotumoural sinus histiocytosis)

si·nus his·ti·o·cy·to·sis with mas·sive lym·phad·e·nop·a·thy

a chronic disease occurring in children and characterized by massive painless cervical lymphadenopathy due to distension of the lymphatic sinuses by macrophages containing ingested lymphocytes, and by capsular and pericapsular fibrosis.

Rosai-Dorfman disease

(rō′zī-dôrf′măn′, -mən)
n.
A chronic disease characterized by painless, massively enlarged cervical lymph nodes whose sinuses contain histiocytes that have engulfed lymphocytes and other cells. Extranodal involvement sometimes occurs.

Rosai-Dorfman disease

A rare, benign idiopathic condition characterised by bilateral cervical lymphadenopathy. It is most common in young black men and women, but may affect other ages and races; it may be extranodal. 

Clinical findings
Massive bilateral cervical lymphadenopathy, fever.

Lab
Leukocytosis, increased erythrocyte sedimentation rate, polyclonal hyperimmunoglobulinaemia.
 
Prognosis
Uncertain; most resolve spontaneously, but some are aggressive and ultimately fatal.

si·nus his·ti·o·cy·to·sis with mas·sive lym·phad·e·nop·a·thy

(sī'nŭs his'tē-ō-sī-tō'sis mas'iv limf-ad-ĕ-nop'ă-thē)
A chronic disease occurring in children characterized by massive painless cervical lymphadenopathy due to distension of the lymphatic sinuses by macrophages containing ingested lymphocytes, and by capsular and pericapsular fibrosis.
Synonym(s): Rosai-Dorfman disease.

Rosai,

Juan, U.S. pathologist, 1941–.
Rosai-Dorfman disease - Synonym(s): sinus histiocytosis with massive lymphadenopathy