sinus histiocytosis with massive lymphadenopathy


Also found in: Acronyms.

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.

sinus histiocytosis with massive lymphadenopathy

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.

sinus histiocytosis with massive lymphadenopathy

Rosai-Dorfman disease Hematology condition most common in young blacks Clinical Massive bilateral cervical lymphadenopathy, fever, leukocytosis, ↑ ESR, polyclonal hyperimmunoglobulinemia Prognosis Uncertain; most resolve spontaneously; some are aggressive and ultimately fatal. Cf Sinus histiocytosis.

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
References in periodicals archive ?
Rosai, "The digestive system manifestations of Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy): Review of 11 cases," Human Pathology, vol.
Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare and benign source of lymphadenopathy of unknown aetiology, first described in 1969 by Rosai and Dorfman (cited by Moore et al.
The ophthalmologic manifestations of sinus histiocytosis with massive lymphadenopathy. Am J Ophthalmol.
Sinus histiocytosis with massive lymphadenopathy (SHML) was first described by Rosai and Dorfman in 1969 as a disease characterized by nodal histiocytic infiltration (2).
Sinus histiocytosis with massive lymphadenopathy (SHML) was initially recognized by Destombes (1) in 1965.
The term sinus histiocytosis with massive lymphadenopathy (SHML) was introduced by doctors Rosai and Dorfman in 1969 as a well-defined clinicopathologic entity that is now widely known as Rosai-Dorfman disease.
The sinus histiocytosis with massive lymphadenopathy (SHML) or RDD is a rare histiocytic disorder of unknown origin which shares several cell markers with JXG.
[sup][11] Germline mutations in SLC29A3 have been reported in rare patients with a wide range of overlapping clinical features and inherited disorders including H syndrome, pigmented hypertrichosis with insulin-dependent diabetes (PHID), Faisalabad histiocytosis, sinus histiocytosis with massive lymphadenopathy, dysosteosclerosis (DSS) and monogenic autoinflammatory syndrome.
Extranodal head and neck sinus histiocytosis with massive lymphadenopathy. Otolaryngol Head Neck Surg 1990;102(6):764-7.
Sinus histiocytosis with massive lymphadenopathy was described by Rosai and Dorfman in 19691 and, therefore, received the eponym Rosai-Dorfman disease (RDD).
In 1969, Rosai and Dorfman (1) described 4 cases of a disease they called sinus histiocytosis with massive lymphadenopathy (SHML).
Sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman disease is a rare benign proliferative histiocytic disease of unknown origin.