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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 (Rosai-Dorfman disease) is a systemic proliferation of cells that resemble the sinus histiocytes of lymph nodes. Whereas initial reports highlighted the often striking cervical adenopathy, more than 40% of patients have extranodal involvement. Cutaneous lesions are the most common form of extranodal disease, but cases that present as cutaneous disease without lymph node involvement are rare. We examined biopsies from three patients with cutaneous lesions of sinus histiocytosis, none of whom had detectable systemic involvement, and compared them to those of two patients whose lymph nodes were involved by the disease. The histology of skin lesions in all five cases showed dense nodular or diffuse infiltrates of "histiocytes," emperipolesis of lymphocytes, neutrophils, and plasma cells. "Histiocytes" were present in lumens of dilated lymphatics. At the peripheries of the lesions were lymphoid aggregates and thick-walled vessels surrounded by plasma cells. Staining with anti-S-100 protein antibody showed marked cytoplasmic positivity in the "histiocytes" in each case. The only features that differed among the two groups were the presence of pseudoepitheliomatous hyperplasia and infiltrates of eosinophils in one case in which the disease was limited to the skin. We believe that cutaneous sinus histiocytosis can be specifically diagnosed by skin biopsy. Because cutaneous sinus histiocytosis may be unfamiliar to general pathologists, dermatopathologists, and dermatologists, cases limited to the skin may be under-recognized.
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PMID:Histologic features of cutaneous sinus histiocytosis (Rosai-Dorfman disease): study of cases both with and without systemic involvement. 140 44

Sinus histiocytosis with massive lymphadenopathy (SHML) is a disorder of unknown origin which is only infrequently associated with lymphoid neoplasms. We report the first two cases of Hodgkin's disease with simultaneous SHML-like changes in the same lymph node.
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PMID:Hodgkin's disease and sinus histiocytosis with massive lymphadenopathy-like changes. 191 95

The production of carcinoembryonic antigen (CEA) by human breast cancer tissue has been studied in relation to the prognosis of 60 patients with breast cancer who were classified in Clinical Stage of UICC I, II and III, and who were treated by radical mastectomy. Tissue CEA was studied in primary tumors using an immunoperoxidase (PAP) method. The obtained results were as followings: 1) Patients who had CEA-negative tumors had significantly higher 10-year survival rates. 2) The positive staining rate of CEA rises as Clinical Stage and metastatic status of lymph nodes advanced. 3) There were significant correlations between staining rate of CEA and classification of histological type and differentiation. 4) There were significant correlations between staining rate of CEA and lymphoid infiltration around main tumor and Sinus Histiocytosis in regional lymph nodes. 5) These results suggest that immunohistological assessment of CEA in breast cancer tissue may provide more precise prognostic information.
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PMID:[A clinicopathological study on carcinoembryonic antigen in breast cancer]. 237 Apr 34

Five cases of Rosai-Dorfman histiocytosis (RDH) (also called Sinus Histiocytosis with Massive Lymphoadenopathy; SHML) have been studied by immunohistochemical methods with heteroantisera and monoclonal antibodies. One case was also studied by Southern blot hybridization analysis with DNA probes specific for T cell receptor beta chain and immunoglobulin heavy chain. Immunophenotyping of large histiocytes, characteristic of RDH, evidenced the presence of S-100 protein and the absence of CD1 and other markers usually found in histiocytes and macrophages. DNA hybridization study showed the absence of clonal T or B lymphoid populations.
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PMID:Immunohistological analysis of Rosai-Dorfman histiocytosis. A disease of S-100 + CD1-histiocytes. 311 Oct 77

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

Nodular skin lesions on the lateral aspects of the legs of a female child were first noticed at the age of 6 days. A biopsy of the lesions was done at the age of 6 months when the child had also developed cervical and inguinal lymphadenopathy and angiomatous lesions on the face. The diagnosis of Rosai-Dorfman disease (RDD; also known as sinus histiocytosis with massive lymphadenopathy, SHML) was made. Increasing respiratory obstruction by lymphoid tissue prompted a 2-week trial with oral prednisolone. A dramatic response occurred, with complete resolution of all clinical findings within 5 days, but with recurrence of lymphadenopathy 6 weeks after stopping with medication. Further observations over the next 3 years established a consistent response to prednisolone and a pattern of steroid dependence.
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PMID:Steroid-responsive Rosai-Dorfman disease. 894 Jul 41

Immunohistochemistry was used to look for the expression of human herpesvirus-6 (HHV-6) antigens in a well characterized series of benign, atypical, and malignant lymphoid lesions, which tested positive for the presence of HHV-6 DNA. A panel of specific antibodies against HHV-6 antigens, characteristic either of the early (p41) or late (p101K, gp106, and gp116) phases of the viral cycle, was applied to the lymphoid tissues from 15 non-Hodgkin's lymphomas, 14 Hodgkin's disease cases, 5 angioimmunoblastic lymphadenopathies with dysproteinemia, 14 reactive lymphadenopathies, and 2 cases of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). In lymphomatous tissues, the expression of late antigens was documented only in reactive cells, and mainly in plasma cells. Of interest, the expression of the early p41 antigen was detected in the so-called "mummified" Reed-Sternberg cells, in two Hodgkin's disease cases. In reactive lymphadenopathies, the HHV-6 late antigen-expressing cells were plasma cells, histiocytes, and rare granulocytes distributed in interfollicular areas. In both cases of Rosai-Dorfman disease, the p101K showed an intense staining in follicular dendritic cells of germinal centers, whereas the gp106 exhibited an intense cytoplasmic reaction in the abnormal histiocytes, which represent the histological hallmark of the disease. The expression of HHV-6 antigens is tightly controlled in lymphoid tissues. The lack of HHV-6 antigen expression in neoplastic cells and the limited expression in degenerating Reed-Sternberg cells argue against a major pathogenetic role of the virus in human lymphomagenesis. The detection of a rather unique pattern of viral late antigen expression in Rosai-Dorfman disease suggests a possible pathogenetic involvement of HHV-6 in some cases of this rare lymphoproliferative disorder.
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PMID:Expression of human herpesvirus-6 antigens in benign and malignant lymphoproliferative diseases. 973 30

Acute renal failure developed in a 57-year-old woman who had Rosai-Dorfman disease diagnosed 1 year previously on a cervical lymph node. Organ imaging showed diffuse masses infiltrating both kidneys. The renal biopsy showed a lymphoplasmacytic and histiocytic process extensively replacing the parenchyma, which is in keeping with Rosai-Dorfman disease of the kidneys. However, the typical lymphophagocytic cells were lacking. This case illustrates that diagnosis of Rosai-Dorfman disease in renal biopsy can be very difficult, requiring both exclusion of many benign and malignant lesions and a high index of suspicion for this condition. In particular, lymphoma was excluded based on the mixed polyclonal composition of inflammatory cells and the absence of atypical lymphoid proliferation. The renal function partially recovered after a course of therapy combining VP-16 (etoposide) and dexamethasone and remained stable over 4-year follow-up. This report emphasizes the importance of early diagnosis and intervention to safeguard renal function in extensive Rosai-Dorfman disease.
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PMID:Acute renal failure in a patient with Rosai-Dorfman disease. 1047 58

We report the cytologic findings of a case of Rosai-Dorfman disease of the breast in a 52-year-old diabetic woman, initially sampled by fine-needle aspiration biopsy (FNA). The patient presented with a 2-week history of a 3 x 2 cm nodule in the mid-upper area of the left breast. A mammogram taken 6 months prior was negative. FNA smears demonstrated lymphocytes, plasma cells, and large pale cells, with enlarged irregular nuclei, admixed with fragments of fibrous tissue and calcific debris. Lymphophagocytosis (emperipolesis) was scarce. Our diagnosis was atypical lymphohistiocytic proliferation. Flow cytometry was negative, but in the face of a strong clinical suspicion of a lymphoid malignancy, excision was performed. The final diagnosis was Rosai-Dorfman disease (RDD). The differential diagnosis of FNA of breast inflammatory lesions with atypical cytology is discussed, with a review of the literature. The early recognition on FNA of the hallmarks of this rare disease should prevent unnecessary radical surgery. Diagn. Cytopathol. 1999;21:287-291.
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PMID:Fine-needle aspiration cytology of Rosai-Dorfman disease of the breast: A case report. 1049 25

Sinus histiocytosis with massive lymphadenopathy (SHML) is a benign, self-limiting condition of unknown etiology, which generally presents as massive bilateral cervical lymphadenopathy. It is important to distinguish SHML from other causes of histiocytosis because of the different treatment modalities. This study was carried out to assess the utility of fine-needle aspiration cytology (FNAC) findings in SHML and to distinguish if from other reactive lymphadenopathies. The lymph nodes in 4 patients (3 male and 1 female) presenting with massive bilateral cervical lymphadenopathy were aspirated. All presented with persistent bilateral cervical lymphadenopathy, polymorphnuclear leukocytosis, and raised erythrocyte sedimentation rate (ESR). Smears showed a reactive lymphoid population consisting of mature lymphocytes, plasma cells, a few polymorphs, and many histiocytes showing emperipolesis. Based on the cytologic and clinical findings, a diagnosis of SHML was made. Histopathology confirmed the diagnosis in all cases. A conclusive diagnosis of SHML can be based on cytology, provided that the cytologic findings are interpreted in the appropriate clinical context. Biopsy can be avoided in these patients. Diagn. Cytopathol. 2000;22:181-185.
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PMID:Cytology of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). 1128 31


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