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 (SHML) is generally regarded as a benign disorder in spite of its propensity to form large masses and to disseminate to both nodal and extranodal sites. Although in most patients the extent of SHML does not appear to determine disease outcome, recent reports have documented that infiltrates of SHML can cause death. To gain perspective on the role of SHML in patient deaths in this population, the authors analyzed the 14 known fatalities in an SHML registry comprising 215 patients. The average age at death was 33 years. Autopsy was performed on eight patients. SHML infiltrates were clearly the cause of death in only two patients (previously reported). In another four cases persistent SHML was prominent at death. The other deaths were the result of complications of defined immunologic abnormalities (five), or of unusual infections (three). The authors have not seen an example of cytologic malignant transformation of SHML.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.