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Query: UMLS:C0019625 (Rosai-Dorfman disease)
 763 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Liver biopsies were studied in 47 cases representing various histiocytosis syndromes. These included 32 cases of hemophagocytic syndrome, 11 cases of Langerhans cell histiocysis (LCH), and 4 cases of other histiocytic disorders. All cases of hemophagocytic syndrome, except one with cytomegalovirus infection, displayed portal lymphohistiocytic infiltrates dominated by T lymphocytes. Activation of the hepatic mononuclear phagocytic system (MPS), evidenced by enlarged von Kupffer cells, some of which were hemophagocytic, was seen in 28 cases of hemophagocytic syndrome. Endothelial enlargement, minor degrees of hepatocellular degeneration, and steatosis were also noted. Ten of the 11 cases of LCH also showed activation of the MPS. It was the only lesion in two biopsies. Seven cases demonstrated nonspecific "triaditis." In three this was associated with cholangiocentric and random acinar histiocytic lesions. Evidence of activation of the MPS was also observed in both cases of Rosai-Dorfman disease and was accompanied by acinar histiocytic lesions in one and triaditis in the other. Likewise, both cases of juvenile xanthogranuloma showed activation of the MPS and focal granulomatous lesions. It is concluded that activation of the MPS is a common feature of liver disease in histiocytosis syndromes and that hepatic enlargement may be the result of this process instead of, or in addition to, the liver lesions known to be featured in these disorders. Hepatic lesions of the various histiocytosis syndromes resemble typical lesions in other sites and, in some instances, are accompanied by nonspecific changes. These nonspecific changes may occur in the absence of lesions that are diagnostic or typical of the particular histiocytosis syndrome. The location and character of hepatic lesions are important factors in the significance of liver involvement.
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PMID:Histopathology of the liver in histiocytosis syndromes. 902 43

Rosai-Dorfman disease (RDD) involves the gastrointestinal tract only in exceptional cases, and this very unusual site of presentation can confuse the pathologist. We present a case of RDD manifesting as an intestinal occlusion caused by colonic diverticulitis. The patient was a 79-year-old man with myelodysplasia, who presented with fever, abdominal pain, and constipation. Colonoscopy revealed sigmoiditis and diverticulosis. Microscopic study of the sigmoid colon surgical specimen showed the histological and immunological features of RDD. No human DNA of herpesvirus types 6 and 8 (HHV6/HHV8), Epstein-Barr virus (EBV), and cytomegalovirus (CMV) was detected in tissue by polymerase chain reaction. Electron microscopic study revealed no microbes or viral particles. Widespread nodal and extranodal RDD occurred, and the patient died 2y after initial surgery. The etiology of RDD is still under debate. We discuss the association of RDD with hematological disorders.
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PMID:Intestinal occlusion caused by Rosai-Dorfman disease mimicking colonic diverticulitis. 1740 Mar 99

Rosai-Dorfman disease (RDD) is a rare benign proliferative disorder of histiocytes in the lymph nodes with or without extranodal involvement. RDD limited to the skin without nodal involvement, known as cutaneous Rosai-Dorfman disease, is very rare. We describe a 34-year-old female with RDD of the skin over the chest. A large nodule with satellite lesions was excised for histopathological examination. Microscopically, there were many large histiocytes (Rosai-Dorfman cells) exhibiting emperipolesis, among many plasma cells, lymphocytes and neutrophils throughout the dermis and the subcutaneous tissue. The histiocytes were immunohistochemically positive for S-100 protein but negative for CD 1a. Physical examination showed no lymphadenopathy or any extra-cutaneous lesions. Serological tests indicated a past infection with Epstein-Barr virus and cytomegalovirus. The diagnosis of cutaneous RDD may be difficult in the absence of associated lymphadenopathy or any specific features of the skin lesion. Hence, not only is histopathological examination required for definitive diagnosis but a high index of suspicion by the clinicians and pathologists is essential to help diagnose this very rare disease.
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PMID:Cutaneous Rosai-Dorfman disease. 1910 29