Immunologic Abnormalities and Their Significance in Sinus Histiocytosis with Massive Lymphadenopathy

Abstract

In a computerized case registry, containing 220 cases of sinus histiocytosis with massive lymphadenopathy (SHML), 23 patients were identified with clinical or routine laboratory findings suggestive or diagnostic of immune dysfunction. We divided the abnormalities into hematologic autoantibodies (nine patients), glomerulonephritis (three patients), Wiskott-Aldrich syndrome (two patients), joint disease (nine patients), unusual infections (three patients), and miscellaneous (six patients). Nine of the patients had more than one finding prompting inclusion in this study. In five patients, at least one abnormality preceded the onset of SHML. A major difference between this subgroup of 23 patients and the remaining registry population was the mortality rate. Ten of the 23 patients have died, and in many cases the cause of death could be linked to the immunologic abnormality. This study establishes an association between SHML and clinically significant immune dysfunction and provides further evidence that this frequently multisystemic disease may be one manifestation of disordered immunity.

Sinus Histiocytosis with Massive Lymphadenopathy and Malignant Lymphoma Involving the Same Lymph Node: A Report of Four Cases and Review of the Literature

“…Patients who have SHML commonly have immunologic abnormalities (5). Possibly, the foci of SHML in the four cases we report are related to immunodeficiency associated with the presence of malignant lymphoma, which predisposed to reactivation of HHV-6 infection.…”

“…Accompanying clinical and laboratory findings include fever, leukocytosis, elevated erythrocyte sedimentation rate, and hypergammaglobulinemia (3). Although fatalities in patients who have SHML rarely have been reported, particularly in patients who have immunologic abnormalities (3,5), most patients who have SHML follow a benign clinical course, often with spontaneous resolution of disease.…”

“…The association of SHML with Hodgkin's disease or non-Hodgkin's lymphoma is rare; nine such cases have been reported in the literature (5)(6)(7)(8)(9)(10)(11). Of these, only four patients had SHML and malignant lymphoma involving the same lymph node simultaneously (10,11).…”

A Rare Case of “Rosai–Dorfman Disease”

“…Recent reports document that infiltrates of SHML can cause death [2][3][4][5][6][7]. Foucar et al [9,10] attribute fatalities associated with SHML to related immunologic disorders. Histiocytes expressed S-100 protein by immunohistochemistry, S-100 protein is found in cytoplasm of many cells of neuroectodermal origin as well as subtypes from the macrophage/histiocyte family which can be demonstrated by immunoperoxidase staining of antibodies directed against S-100 protein.…”

Rosai-Dorfman disease in a 12-year-old Nigerian male

“…Patients with clinically apparent immunological disorders have a higher mortality. There also appears to be a familial relationship with regard to RDD: two sets of twins, two other sets of siblings and four individuals of blood relatives were documented by Foucar [8] in 1984. RDD has also been presumed to be triggered by infections caused by viruses such as various herpes viruses, the Epstein-Barr virus, cytomegalovirus, Brucella and Klebsiella.…”