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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 with massive lymphadenopathy is an idiopathic, histologically distinctive disorder that characteristically produces painless cervical lymphadenopathy. Other node groups are frequently involved and mass lesions in extranodal sites occur in approximately 30% of patients. In most cases the disease is chronic and the course is often characterized by exacerbations and remission. We report the case of a 66 years old white woman affected by sinus histiocytosis with massive cervical and inguinal lymphadenopathy. Subcutaneous nodules appeared in her left arm and low back region with the histological features of sinus histiocytosis. Cellular infiltrate was composed predominantly of histiocytes aggregating in granuloma like clusters. These cells had large round or kidney shaped nuclei; cytoplasm was abundant, vacuolated, often containing phagocytized lymphocytes. Multinucleated cells were also observed; reactive plasmocytosis was present in the nodule periphery. During the follow-up the patient maintained good physical conditions, despite a moderate weight loss. The size of her nodules regressed within one year. Immunologic alterations, consisting of polyclonal hypergammaglobulinemia and persistent inversion of T4/T8 lymphocyte ratio, were observed. Sinus histiocytosis usually offers a good prognosis; in our patient infective and/or autoimmune complications could be favoured by advanced age, extranodal involvement and immunological depression.
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PMID:[Skin involvement in Rosai-Dorfman disease. Description of a case]. 280 94

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

The association of adenopathies, having sometimes a tumoral aspect, and a polyclonal hypergammaglobulinemia in the blood can be observed in various illnesses. In all these diseases the increase in the volume of the lymph nodes is partly due to the hyperplasia of the lymphoid B regions. Lymph node biopsy allows a precise diagnosis to be made, providing a thorough analytical study of the histopathological lesions is performed. In this first part, the histopathological lesions of benign lymphadenitis, having a pseudo-tumoral aspect from a clinical view, are described. The following are thus successively studied: sarcoidosis; acquired toxoplasmosis; the various aspects which can be attributed to infectious mononucleosis; vaccinal reactions; the Destombes-Rosai-Dorfman syndrome or massive lymphadenopathy by sinusal histiocytosis with hemophagocytosis; angiofollicular lymphoid hyperplasia or pseudo-tumour of Castleman; angio-follicular and plasmocytic polyadenopathy; the adenopathies of collagenosis and those due to protein deposits. For each disease, the morphological data are discussed and compared to clinical state and biological modifications.
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PMID:[Reactive hyperplasia and pseudo-lymphomas with hypergammaglobulinemia. I. Benign lymphadenopathies]. 636 8

Rosai-Dorfman disease, first described in 1969, is a rare idiopathic histioproliferative disease affecting the lymph nodes. Typical clinical features include bilateral painless lymphadenopathy, fever and polyclonal hypergammaglobulinemia. In approximately 43% of cases, extranodal sites may be involved and occasionally represent the initial or sole manifestation of the disease. Central nervous system manifestations are exceedingly rare, and only 17 cases have been recorded, among which there are merely seven isolated intracranial cases without concurrent nodal or other extranodal involvement. Herein, we report a 38-year-old male presenting with generalized tonic-clonic seizure and radiological findings indicative of meningioma. Complete physical examinations and laboratory surveys demonstrate the absence of involvement at other body sites. Microscopically, the lesion consists of proliferative histiocytes exhibiting emperipolesis coupled with the characteristic cytoplasmic staining against S-100 protein. The differential diagnosis of polymorphic inflammatory meningioma-mimicking masses is discussed, and a review of previously reported intracranial Rosai-Dorfman disease is presented.
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PMID:Isolated intracranial Rosai-Dorfman disease: case report and literature review. 970 47

Presented is a case of a 31-year-old woman with cutaneous Rosai-Dorfman disease, which is a rare, benign, generally self-limited histiocytic proliferative disorder that is characterized by painless lymphadenopathy, fever, neutrophilia, an elevated erythrocyte sedimentation rate, and polyclonal hypergammaglobulinemia. It primarily involves lymph nodes but can affect extranodal sites such as the skin, where it typically appears as indistinct erythematous papules and nodules. On histopathologic examination, emperipolesis is a consistent finding. The etiology is unknown, and treatment is based on clinical manifestations.
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PMID:Cutaneous Rosai-Dorfman disease. 1574 82

Rosai-Dorfman disease (RDD) is a rare, sporadic histiocytic disorder characterized by painless but protracted lymphadenopathy. Its etiology remains unclear. The observation of congenital disease and reports of familial cases with seven pairs of siblings including three sets of identical twins suggests a genetic predisposition in some patients with this condition. We now report two brothers of consanguineous Palestinian parents, whose lymphadenopathy, lymph node histology, and polyclonal hypergammaglobulinemia indicated RDD. The presence of intrauterine fractures, short stature, and sensorineural hearing impairment suggested a rare familial form of the disorder. Moynihan et al. recently described a Pakistani family with a familial histiocytic disorder highly reminiscent of the brothers reported here, whose lymph node morphology was apparently consistent with RDD as well. The presence of sensorineural deafness, short stature, and joint contractures, however, suggested a separate, rare autosomal recessive syndrome referred to as Faisalabad histiocytosis, after the family's place of origin. We believe that the brothers described here represent a second family with Faisalabad histiocytosis, which mimics RDD histologically.
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PMID:Faisalabad histiocytosis mimics Rosai-Dorfman disease: brothers with lymphadenopathy, intrauterine fractures, short stature, and sensorineural deafness. 1615 31

An 18-year-old man presenting with paraplegia due to spinal epidural infiltration at D6-D7 underwent laminectomy and spinal decompression followed by near-complete resolution of neurological symptoms; but this resolution was followed by progressively enlarging cervical lymph nodes. Leucocytosis, an elevated erythrocyte sedimentation rate, a marked polyclonal hypergammaglobulinemia and a moderate hepatosplenomegaly were found. The diagnosis of sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease was established histologically by lymph node biopsy about 2 years after presentation.
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PMID:A young male with paraplegia, massive cervical adenopathy and nondiagnostic biopsy specimens. 1759 39

Rosai-Dorfman disease, that is, sinus histiocytosis with massive lymphadenopathy is a benign systemic proliferative disorder of histiocytes. The typical clinical presentation of the disease includes bilateral painless massive lymphadenopathy, fever and polyclonal hypergammaglobulinemia. Extranodal involvement is present in only a few cases and skin lesions are the most common form of extranodal disease. However, purely cutaneous Rosai-Dorfman disease is uncommon. In this study, we describe a 10-year-old child presenting with bilateral ocular involvement.
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PMID:Extranodal manifestation of Rosai-Dorfman disease with bilateral ocular involvement. 2189 50

Rosai-Dorfman disease (RDD)-sinus histiocytosis with massive lymphadenopathy-represents a peculiar proliferation of histiocyte-like cells in patients. The condition was described by Rosai and Dorfman in 1969, after examining 4 cases, as an idiopathic histiocytic disorder. In 1972, they studied an additional 30 cases of patients with RDD. A histioproliferative disorder, RDD is characterized by bilateral, painless, cervical lymphadenopathy in 81% of patients. Fever, leukocytosis, elevated sedimentation rate, and polyclonal hypergammaglobulinemia may also be found. In 30% of patients, extranodal involvement is present and may include the skin, eye orbit, upper respiratory tract, or testes. Cases involving the central nervous system are rare and account for < 5% of patients with RDD. We report on a 78-year-old woman presenting with new-onset headache, dizziness, and imbalance, which had been present for a few weeks prior to admission. Magnetic resonance imaging of the brain showed 2 enhancing lesions within the right and left cerebellar hemispheres. Biopsy of the mass demonstrated a lymphohistiocytic infiltrate involving the cerebellum with foci of emperipolesis (phagocytosed lymphocytes). The adjacent cerebellum showed myelinated nerve fibers with reactive gliosis. A thorough work-up and histopathologic exam of the biopsied mass demonstrated lymphohistiocytic infiltrate with foci of emperipolesis (phagocytosed lymphocytes) consistent with RDD. Other differential considerations, such as primary or secondary neoplasms, infections, lymphoproliferative disorders, granulomatoses, Langerhans cell histiocytosis, and lymphocyte-rich meningioma were ruled out by additional histopathologic exam.
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PMID:Case report of intracranial Rosai-Dorfman disease. 2414 94

Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is a benign lympho-histiocytic proliferative disorder initially described with bilateral painless lymphadenopathy (90 %), fever, leukocytosis, elevated ESR, anemia, and polyclonal hypergammaglobulinemia (90 %). Extranodal forms occur in 43% of cases, the skin being the most common site. Around 10% of patients have skin lesions and in 3%, the disease is limited exclusively to the skin. Here, we report a male patient who presented with pure cutaneous lesions which mimic eruptive xanthoma clinically. However, the diagnosis was established histo pathologically. So, high level of clinical suspension is critical to avoid missing such cases.
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PMID:A new presentation of isolated cutaneous Rosai-Dorfman disease: Eruptive xanthoma-like lesions. 3033 58


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