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

The histiocytoses, whether reactive or neoplastic, can be related histologically and immunophenotypically to their normal counterparts within the histiocytic system. This system has two subsets: The dendritic (antigen-presenting) cells and the phagocytic histiocytic (antigen-processing) cells. Dermatopathic lymphadenitis and Langerhans cell histiocytosis (histiocytosis X) are reactive proliferative disorders of dendritic cells. Malignancies of dendritic cells exist, but they are very rare. Benign proliferations of phagocytic histiocytes include the hemophagocytic syndromes, both familial and reactive, as well as sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and histiocytic necrotizing lymphadenitis (Kikuchi's disease). Neoplasms of phagocytic histiocytes include acute monocytic leukemia and the very rare entities, malignant histiocytosis and true histiocytic lymphoma. The latter must be distinguished from sinusoidal, large cell anaplastic lymphomas.
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PMID:The histiocytoses: clinical presentation and differential diagnosis. 215 Mar 25

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

Nosological classification of sinus histiocytosis with massive lymphadenopathy (SHML; Rosai-Dorfman disease) is difficult, and the normal cellular counterpart of Rosai-Dorfman (RD) cells is uncharacterised. The peculiar S-100+ phenotype of RD cells suggests a relationship with the dendritic cell family. Recent investigations have revealed cathepsin E to be selectively concentrated in antigen-presenting cells, whereas cathepsin D was found to be expressed in cells of macrophage lineage. Cathepsin D and E distribution was investigated by immunohistochemistry in a series of SHML biopsies and in two types of dendritic cell proliferative lesions: dermatopathic lymphadenitis (DL) and Langerhans' cell histiocytosis (LCH). In SHML biopsies, RD cells and monocyte-related elements of the sinuses and pulp coexpressed cathepsin D and E. LCH cells also stained for both these aspartic proteinases. Conversely, in DL cathepsin E and D were localised to separate cells that resembled Langerhans' cells (LC) or macrophages, respectively, in morphology and distribution. Our data outline the peculiar immunophenotype of RD and LCH cells and suggest that caution should be exercised in the identification of their normal cellular counterpart. The common expression of cathepsin D and E and of S-100 protein suggests some phenotypic overlap between SHML and LCH cells, despite their striking morphological divergence.
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PMID:Cathepsin D and E co-expression in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and Langerhans' cell histiocytosis: further evidences of a phenotypic overlap between these histiocytic disorders. 805 53

Sinus histiocytosis with massive lymphadenitis or Rosai-Dorfman disease (RDD) is a rare, benign, proliferative histiocytic disease of unknown origin. It predominately affects the lymph nodes The skin is the extranodal organ most frequently involved, although a few patients present with only lesions of the skin and soft tissues, without involvement of the lymph nodes (purely cutaneous RDD). Ocular involvement is rare, and generally localized to the eyelid. We report an unusual case of cutaneous sinus histiocytosis without nodal involvement and with bilateral uveitis in a girl. As far as we know, this is the first case reported in which cutaneous and ocular RDD occurred without nodal involvement.
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PMID:Cutaneous sinus histiocytosis and chronic uveitis. 1108 66

This study was undertaken to document the grey-scale and power Doppler sonographic features of cervical lymphadenopathy in Kikuchi's disease (histiocytic necrotising lymphadenitis), Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy), Sjogren's syndrome and systemic lupus erythematosus (SLE), which have not been reported in the literature. A retrospective review of the grey-scale and power Doppler sonograms of the cervical lymph nodes in nine patients was conducted (Kikuchi's disease, n = 3; Rosai-Dorfman disease, n = 1; Sjogren's syndrome, n = 1; SLE, n = 4). Lymph nodes were proven to be pathologic by fine-needle aspiration cytology (FNAC). On grey-scale ultrasound (US), lymph nodes were assessed by their distribution, size, shape, echogenicity and internal architecture. The vascular pattern of the lymph nodes was assessed with power Doppler sonography. US features of the lymph nodes were compared to those of metastatic and reactive nodes. In Kikuchi's disease, Rosai-Dorfman disease, Sjogren's syndrome and SLE, the distribution of lymph nodes is similar to that of reactive nodes. Most of the lymph nodes are enlarged with a maximum transverse diameter greater than or equal to 10 mm (83.3 to 100%). In Kikuchi's disease, lymph nodes have grey-scale and Doppler appearances similar to reactive nodes. However, lymph nodes in Rosai-Dorfman disease, Sjogren's syndrome and SLE show similar grey-scale and Doppler features to metastatic nodes. There is no specific US feature to characterise lymphadenopathy from these four miscellaneous causes. Definitive diagnosis should still be based on cytology and histology, and US can help in guiding FNAC for a more accurate cytologic examination.
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PMID:Grey-scale and power Doppler sonography of unusual cervical lymphadenopathy. 1512 Dec 46

Here we describe a case of Rosai-Dorfman Disease (RDD) in a 25-year-old female patient from Turkey who was previously misdiagnosed with Toxoplasma Lymphadenitis, and review the manifestations and treatment of this rare entity. To the best of our knowledge this is the third description of RDD [Sinus Histiocytosis with Massive Lymphadenopathy (SHML)], involving bilateral cervical lymphadenopathy and nephromegaly previously misdiagnosed as Toxoplasma Lymphadenitis. Representative clinical, radiographic and histological findings are presented. Its etiology, diagnosis and management are also reviewed. Sinus Histiocytosis with Massive Lymphadenopathy is a rare disorder of unknown etiology, usually associated with lymph node enlargement in various superficial or deep sites. The key histologic feature of SHML is the presence of various numbers of large, pale histiocytic cells that contain within their cellular borders apparently engulfed lymphocytes (emperipolesis); these distinctive large, pale cells are S-100 protein positive CD-68 positive and CD1a negative by immunostaining. According to the literature the most effective treatment found was surgical debulking.
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PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman's disease) previously misdiagnosed as Toxoplasma Lymphadenitis. 1529 64

Lymphadenomegaly is a common sign of benign and malignant disorders. In our practice, only every fifth patient is found to have primary or secondary nodal malignancy. Benign disorders, including banal infections and other non-neoplastic conditions, however, cause most of the cases. Among these, there are some rare entities, resulting in persistent lymphadenopathy that may cause differential diagnostic problems in the daily practice. We report here three patients, having Rosai-Dorfman disease, multicentric Castleman's disease and Kikuchi's lymphadenitis, who exemplify such cases. Our purpose with this presentation is to emphasise importance of vigorous co-operation between clinicians and pathologists. In general, it is imperative to prefer specialised haematopathological laboratories that may facilitate proper diagnosis.
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PMID:Uncommon lymphadenopathies of immunopathogenesis can be misinterpreted as malignant diseases. 1716 Jun 84

Peripheral T-cell lymphomas are a heterogeneous group that often requires the use of ancillary testing for accurate diagnosis. This is particularly applicable to the diagnosis of angiommunoblastic T-cell lymphoma (AITL) and peripheral T-cell lymphoma, unclassified (PTCLU), because of their histologic and immunophenotypic overlap with reactive lymphoid proliferations. Recently, immunohistochemistry for programmed death-1 (PD-1), a marker of follicular helper T cells, was shown to be sensitive in the detection of AITL and PTCLU. The sensitivity of this marker in reactive entities, however, has not been adequately evaluated. We confirm that PD-1 staining is a highly sensitive marker in the diagnosis of peripheral T-cell lymphomas: increased extrafollicular PD-1-positive cells were seen in 93% (76/82) of AITL, 62% (16/26) of PTCLU, and 11% (2/18) of anaplastic-lymphoma-kinase (ALK)-negative anaplastic large-cell lymphomas. The majority of reactive lymphadenopathies including Cat-scratch disease, Kikuchi lymphadenitis, Castleman disease, and reactive follicular hyperplasia showed no PD-1 staining outside follicles. Some reactive lymph nodes, showed increased extrafollicular PD-1-positive cells in a pattern similar to AITL and PTCLU, and include progressive transformation of germinal centers, viral lymphadenitis (Epstein-Barr virusand human immunodeficiency virus) and Rosai-Dorfman disease. This study shows that PD-1-positive cells may be increased in a number of settings other than T-cell lymphomas. We conclude that staining for PD-1 in reactive and atypical lymphadenopathies should be interpreted with caution and in the context of other ancillary immunophenotypic and molecular studies before a diagnosis of AITL or PTCLU is entertained.
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PMID:PD-1 expression in T-cell lymphomas and reactive lymphoid entities: potential overlap in staining patterns between lymphoma and viral lymphadenitis. 2008 61

Rosai-Dorfman Disease (RDD) is a rare benign disease characterized by sinus histiocytosis with massive lymphadenopathy. RDD can be differentiated from other types of histiocytosis by immunochemical analysis, as RDD is positive for S100. Conversely, toxoplasmosis lymphadenitis is characterized by clusters of epithelioid histiocytes in lymphoid tissue, with mixed lymphocytic and immunoblastic cell populations. The serology data could help in diagnosing toxoplasmosis lymphadenitis, as the toxoplasma IgG should be positive. Here we present the rare case of a 73-year-old woman who presented with a left parotid mass and multiple neck lymphadenopathy that initially had been diagnosed as toxoplasmosis lymphadenitis from a positive result on serology examination, but was finally proven to be RDD based on immunochemical results. We also discuss the relationship between RDD and toxoplasmosis in this case.
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PMID:Toxoplasma IgG expressed in a patient with Rosai-Dorfman disease. 2063 40


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