Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman disease) in an HIV-positive patient

Abstract

We report a case of a 31-year-old HIV infected black female, who presented with asymptomatic generalized lymphadenopathy. Three particularly enlarged lymph nodes were biopsied (2 cervical and 1 axillary). The histological picture was consistent with a diagnosis of sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease. Large histiocytes, positive for a variety of macrophage markers and for the S-100 protein, were observed in the distended sinuses. A few hyperplastic follicles, such as usually seen in HIV-infection-associated lymphadenopathy, were present at the periphery of one lymph node. No infections agent besides HIV could be detected by histological or microbiological analysis or by in situ hybridization. This is the first reported case of SHML associated with HIV infection. The possible relationship between the two diseases is discussed.

Fatal histiocytic proliferative disorders in paediatric HIV infection with cytomegalovirus end‐organ disease

“…Involvement of abdominal organs (liver, spleen, kidneys) is rare (<3%) and associated with progressive, non‐remitting disease (McClain et al , 2004). Four cases of HIV infection associated with RDD have been reported in the literature, none in children (Delacretaz et al , 1991; Dickson‐Gonzalez et al , 2008; Perry et al , 1998). Persistent infection with herpes viruses has been implicated in pathogenesis (McClain et al , 2004).…”

Is it a Metastatic Disease

“…8,22,23 When the disease arises in the lymph nodes, the collagen capsule thickens and becomes fibrotic, and the subcapsular sinus expands. Patients with RDD often experience fever, fatigue, night sweats, and other such generalized symptoms.…”

“…5 RDD typically occurs in children and young adults, and the ratio of male to female patients is 1.4:1; furthermore, it is mainly observed in individuals of African and European descent, rather than in Asian populations. Some researchers have reported that it may be related to infections by the Epstein-Barr virus or human herpesvirus (HHV-6), 8,9 whereas some consider it to be a genetic disease and that it represents a dysfunction of the immune system. A few reports have described RDD affecting 2 members of the same family.…”

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and cirrhosis of the liver: A case report and literature review

“…[3] SHML has been found to occur concomitantly with other diseases such as diffuse large B-cell lymphoma, [1] HIV infection, [2] varicella zoster infection and autoimmune lymphoproliferative syndrome. [7] Delacretaz et al [8] reported the first case of sinus histiocytosis and HIV, in a 31-year-old black woman with HIV infection who presented with symptomatic generalised lymphadenopathy. Since then a few other such cases have been reported, including that by Perry et al [9] in 1998.…”