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

Sinus histiocytosis is a well-recognized condition that mainly affects children in the first decade of life. Although this usually benign disease often features massive cervical lymphadenopathy, numerous extranodal sites of involvement can be seen. We describe a new presenting manifestation for sinus histiocytosis. A 13-month-old infant developed a rapidly growing unilateral epibulbar tumor that involved the cornea. A complete physical examination revealed only mild inguinal lymphadenopathy. A diagnosis of sinus histiocytosis was made by histologic examination of the lesion after surgical excision.
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PMID:Sinus histiocytosis presenting as an epibulbar mass. A clinicopathologic case report. 317 54

Sinus histiocytosis with massive lymphadenopathy is a distinct clinical entity, with ophthalmic involvement in 10% of patients. Orbital as well as eyelid lesions have been described as part of the extra-lymph node involvement of this disorder. We recently examined a young boy with sinus histiocytosis with a bulbar conjunctival mass and chronic dacryocystitis.
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PMID:Ocular involvement in sinus histiocytosis with massive lymphadenopathy. 319 Oct 84

Sinus histiocytosis with massive lymphadenopathy (SHML) is primarily a disease of children and young adults in which there is a pronounced and persistent cervical lymph node enlargement that usually is bilateral and is accompanied by fever. The histology, which varies according to the stage of the disease, is characterized by an exuberant intrasinusoidal histiocytic proliferation. The present case involves a 4-year-old girl who had several episodes of upper respiratory infection and otitis media; subsequently, a walnut-sized enlargement developed in the left anterior portion of the neck. Results of a physical examination were essentially normal. A laboratory work-up was noncontributory. Serologic tests for toxoplasmosis, infectious mononucleosis, and cat-scratch disease were negative. Immunoelectrophoresis disclosed normal values for IgG, IgM, IgA, and IgE. The histopathology was characteristic of SHML. The lymph node demonstrated pericapsular and capsular fibrosis and widely dilated subcapsular, trabecular, and medullary sinuses packed with histiocytes and plasma cells. "Lymphophagocytosis" and large atypical histiocytes resembling Reed-Sternberg cells were noted. Immunohistochemistry demonstrated a polyclonal population of plasma cells mostly stained with rabbit anti-human igG. The cytoplasm of the histiocytes, having ingested lymphocytes, was positively stained for IgG. Other groups of lymph nodes were affected during the next several months. The patient's condition has now been followed for 2 years, and the lymphadenopathy has almost completely regressed. The site distribution of the head and neck extranodal manifestations of SHML was analyzed in 54 cases.
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PMID:Sinus histiocytosis with massive lymphadenopathy (Destombes-Rosai-Dorfman syndrome) occurring as a single enlarged submandibular lymph node: a light and immunohistochemical study with review of the literature. 330 80

Sinus histiocytosis with massive lymphadenopathy (SHML) was originally defined as a relatively specific benign pseudolymphomatous disorder. Although the etiology remains unknown, the spectrum of SHML has been expanded to include predominance of extranodal disease in some patients, clinically significant immunologic abnormalities in 10% of patients, and fatal outcome in 7% of patients. We report the rare occurrence of SHML in identical twins; to our knowledge, SHML in identical twins has been reported only once previously. The two patients described are also unusual because of the predominance of retroperitoneal disease with minimal peripheral adenopathy. After a seven-year clinical course, one twin died of extensive retroperitoneal disease, liver failure, bleeding diathesis, and seizure disorder. The other twin is alive after a six-year course of progressive retroperitoneal disease.
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PMID:Sinus histiocytosis with massive lymphadenopathy. Occurrence in identical twins with retroperitoneal disease. 334 28

A 72-year-old woman presented with the sudden onset of multiple, large, firm, subcutaneous nodules in her right arm and left thigh. Biopsy of the lesions revealed a septal and lobular panniculitis of unknown cause. The lesions regressed spontaneously without therapy over a period of a few weeks. A follow-up review of the microscopic slides from the biopsy disclosed a lymphohistiocytic and plasma cell proliferation with lymphophagocytosis by histiocytes, the characteristic morphological features of sinus histiocytosis with massive lymphadenopathy. This diagnosis was further supported by the immunocytochemical demonstration of S100 protein reactivity within the histiocytic cells. A seven-year follow-up has failed to reveal any evidence of recurrence or progression of the disease. Sinus histiocytosis with massive lymphadenopathy should be entertained in the differential diagnosis of cutaneous lesions presenting with septal and lobular panniculitis.
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PMID:Histiocytic lymphophagocytic panniculitis. An unusual extranodal presentation of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). 340 Oct 30

Sinus histiocytosis with massive lymphadenopathy is a rare clinicopathologic entity that affects mainly children and young adults. Although the lymph nodes are the most common sites of involvement, extranodal infiltrates may arise in the orbits, skin, upper respiratory tract, and bone, as well as other organs. Approximately 10% of patients have had cutaneous infiltrates, but very few of them have had lesions limited to the skin. We treated a 15-year-old female with cutaneous nodules as the sole manifestation of the disease.
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PMID:Sinus histiocytosis clinically limited to the skin. 342 58

Sinus histiocytosis massive lymphadenopathy (SHML) syndrome with hepatic involvement, occurring in a seven-year-old black female, is reported. Morphologic characterization of the hepatic lesion is accomplished utilizing conventional light, fluorescent and electron microscopy, and histochemical techniques and by comparing and contrasting the findings with those in cases of familial erythrophagocytic lymphohistiocytosis (FEL) and virus-associated hemophagocytic syndrome (VAHS). The histiocytic proliferation in the liver in SHML differs by showing: (a) an intralobular distribution with portal sparing; (b) marked steatosis; and (c) lipofuscinosis. The aforesaid intralobular distribution and the accompanying hypertrophy and hyperplasia of Kupffer cells, as well as commonalities of steatosis and lipofuscinosis, and, to a lesser extent, erythrophagocytosis and siderosis, suggest a histogenesis from Kupffer cells. The histochemical finding of fatty acid peroxides and both fluorescent microscopic and histochemical evidence of lipofuscin inclusions, a by-product of lipid peroxidation, in Kupffer cells provide at least a theoretical basis for both erythrophagocytosis and proliferation eventuating in intralobular histiocytosis.
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PMID:Sinus histiocytosis massive lymphadenopathy syndrome: histogenesis of the hepatic lesion. 360 23

Sinus histiocytosis with massive cervical lymphadenopathy (SHML) was originally described in 1969 as a benign clinicopathologic entity characterized by massive bilateral cervical lymphadenopathy, fever, leukocytosis, elevated ESR, and hypergammaglobulinemia, usually occurring within the first two decades of life. We present an illustrated case of an elderly patient with polyclonal hypergammaglobulinemia and a 2-year history of multilobulated cervical and submandibular lymphadenopathy. The etiology and pathogenesis of SHML are not known. Diagnosis requires lymph node biopsy to exclude other causes of cervical lymphadenopathy such as malignant lymphoma, malignant histiocytosis, metastatic carcinoma, and tuberculous lymphadenitis. Histologic examination shows marked dilatation of subcapsular and medullary lymph node sinuses containing large, foamy or vacuolated histiocytes. Although no curative treatment is known, corticosteroids, radiation therapy, vinblastine and oral cyclophosphamide, and surgery have been used to palliate constitutional symptoms and mechanical obstruction from massive lymphadenopathy. Since one third of SHML patients have evidence of disease for 5 years, and a mortality rate of 7% exists with benign histologic disease, all patients with SHML should be carefully screened for evidence of immunodeficiencies that may precipitate a fatal outcome.
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PMID:Sinus histiocytosis with massive cervical lymphadenopathy. Case report and literature review. 374 Jul 6

A single osteolytic bone tumour with a cellular composition similar to that of the extra-nodal localization of Sinus Histiocytosis with Massive Lymphadenopathy although with greater cellular atypicality is described. This histological similarity suggests the possible occurrence of isolated bone involvement of Rosai-Dorfman disease, with atypical cytology but benign evolution, which has not been reported in the literature.
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PMID:Atypical lymphohistiocytic bone tumour (osseous variant of Rosai-Dorfman disease?). 393 59

We report on a 19-year-old girl suffering from cutaneous manifestation of sinus histiocytosis associated with massive lymphadenopathy (SHML) (Rosai-Dorfman syndrome); as SHML had first appeared at her 7th month of life, the disease had taken an uncommonly long period of time. At the age of 15, cutaneous manifestations developed on cheeks and left upper arm which histologically showed the same infiltrate as affected lymph nodes usually do in SHML. So far, participation of the cutaneous system in SHML has been observed in only 12 cases.
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PMID:[Skin manifestations in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman syndrome)]. 398 32


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