Sinus Histiocytosis with Massive Lymphadenopathy: Virological, Immunological and Morphological Studies

Abstract

The histiocytes from a patient with sinus histiocytosis were tested for the presence of Epstein-Barr viral nuclear antigen (EBNA) before and after in vitro growth. On both occasions the histiocytes as well as the lymphocytes were EBNA negative. Likewise the profile of EBV viral capsid antibody (VCA) suggests that this virus is unlikely to be directly involved in the development of the disease. The maintenance of an unusually high level of antibodies to EBV and measles, both viruses which produce persistent infections, indicates that the humoral immune response is active. That a normal humoral response occurred in response to mumps virus during the same period suggests that a deficiency in the cell mediated immune response to the persistent viruses might be the reason for the abnormally high antibody levels. The fresh as well as the cultured histiocytes had only Fc receptors. The affected lymph node lymphocytes developed unusual large inclusion bodies following in vitro culture.

Histologic Features of Sinus Histiocytosis With Massive Lymphadenopathy in Patients With Autoimmune Lymphoproliferative Syndrome

“…32,33 In addition, a variety of infectious agents, including herpes group viruses, Brucella and Klebsiella have been postulated to be involved. 19,22 Our histologic observations suggested to us the possibility of an association between ALPS and SHML. Both disorders share many clinical features, including a young age at presentation, lymphadenopathy, and hypergammaglobulinemia.…”

Sinus histiocytosis with massive lymphadenopathy in three brothers

“…Rosai‐Dorfman disease (Sinus Histiocytosis with Massive Lymphadenopathy; SHML), is a rare cause of lymph node enlargement in children 1 . It consists of chronic massive enlargement of cervical lymph nodes frequently accompanied by fever, leukocytosis, elevated erythrocyte sedimentation rate (ESR), and hyperglobulinemia 2,3 . Extranodal sites are involved in approximately 25% of patients 3 .…”

Sinus Histiocytosis with Massive Lymphadenopathy: A Case Report with Pleural Effusion and Cervical Lymphadenopathy

“…Most patients with SHML tend to have a chronic massive enlargement of cervical lymph nodes frequently accompanied by fever, elevated ESR, neutrophilia, polyclonal gammopathy (9). Our patient had no history of fever and laboratory investigations showed a normal complete blood count, ESR, immunoglobulin levels.…”