Gene/Protein Disease Symptom Drug Enzyme Compound
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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 (SHML) is a rare benign disease of unknown etiology. It is rarely associated with malignant lymphoma. This report documents the first case of a T-cell lymphoma, which developed in a patient with a 10-year history of SHML. The disease was complicated by hypereosinophilia and massive retroperitoneal lymphadenopathy. Histological examination of a cervical lymph node biopsy during the terminal phase identified a lymphoma composed of cells with morphological plasmacytoid features. Ultrastructurally, the tumor cells showed poorly developed cytoplasm, nuclei with peripheral chromatin clumping, and inconspicuous nucleoli. Cytogenetic studies showed two related clones. On immunohistochemical staining tumor cells were positive with monoclonal antibodies (mAb) CD3 and CD45RO. Southern blotting analysis identified clonal rearrangements in the T-cell receptor (TCR) alpha, beta and gamma genes. Thus, T-cell lineage of the tumor cells was established. In situ hybridization of interleukin-2 (IL-2) and interleukin-5 (IL-5) cDNA probes on tissue sections identified the synthesis of IL-5 by the eosinophils, suggesting an autocrine pathway of eosinophilopoiesis leading to hypereosinophilia in this patient.
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PMID:Morphological, ultrastructural, and genetic characterization of an unusual T-cell lymphoma in a patient with sinus histiocytosis with massive lymphadenopathy. 786 28

Rosai-Dorfman disease is rare. Two patients, one with nodal and another with extra-nodal disease, are presented. The clinical and pathological features of the two cases are typical of the disease. Because the disease can present with a diverse picture, it can be easily misdiagnosed. It is therefore suggested that physicians and paediatricians should exercise more vigilance in order to detect more cases, particularly in the early stages, for better understanding of the natural history of this disease about which little is known at this stage.
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PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) in Liberia. 789 9

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

We describe one case of Rosai-Dorfman disease (RDD) localized in the breast and one case of breast malacoplakia (MPK) both diagnosed by fine-needle aspiration cytology (FNAC). In RDD the cytologic features were lymphocytophagocytosis, large histiocytes with slight nuclear atypia, and lymphohistiocytic aggregates in a polymorphous background which included frequent plasma cells. In MPK the key diagnostic feature was the presence of Michaelis-Gutmann bodies (M-G bodies), in various stages of evolution, intermingled with vacuolized medium-sized histiocytes, other inflammatory cells, and debris. Pap staining is the technique of choice to show lymphocytophagocytosis and M-G bodies and there is no need to resort to special staining methods. RDD and MPK share a common cytologic background: the presence of numerous benign reactive histiocytes, either engulfing apparently intact lymphocytes, or phagocytizing M-G bodies. The cytologic findings seen in both cases suggest that the presence of histiocytes in a breast aspirate is far from being nonspecific and can represent an important lead to disparate diseases such as RDD and MPK.
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PMID:Malacoplakia and Rosai-Dorfman disease: two entities of histiocytic origin infrequently localized in the female breast--the cytologic aspect in aspirates obtained via fine-needle aspiration cytology. 814 49

A 57-year-old man presented with a history of nasal obstruction of five to six years duration. 'Nasal polyps' were removed on several occasions. He had previously had an episode of paraplegia which resolved after the removal of a spinal tumour. Histology from both sites was thought to represent a malignant fibrous histiocytoma. On presentation the patient had computed tomographical (CT) evidence of extensive ethmoidal disease, with threatened intracranial extension. He also had evidence of lung and retroperitoneal disease with pancreas and kidney involvement. The ethmoidal disease was considered potentially lethal and therefore a craniofacial resection was performed. Review of all the histology revealed that the diagnosis was extranodal Rosai-Dorfman disease (sinus histiocytosis). The patient's course is described, and the literature on this disease of unknown aetiology is reviewed.
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PMID:Rosai-Dorfman disease of the paranasal sinuses. 816 19

Sinus histiocytosis with massive lymphadenopathy was first described in 1969 by Rosai and Dorfman. The typical clinical characteristics of this disease include painless cervical lymphadenopathy, fever, and weight loss. The condition can present with an extranodal mass in about 25% of patients, and isolated masses without lymph node involvement occur rarely. The authors describe a 5-year-old boy with cavernous sinus syndrome due to an isolated extranodal form of sinus histiocytosis with massive lymphadenopathy in the temporal fossa. Several cases of this disease involving the central nervous system are reviewed. The histopathological and magnetic resonance imaging characteristics are discussed.
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PMID:Isolated extranodal intracranial sinus histiocytosis in a 5-year-old boy. Case report. 841 Feb 58

The authors describe a 20-year-old man who initially developed an intradural mass in the upper cervical region and subsequently presented with nasal/paranasal sinus and posterior fossa masses, well demonstrated by CT and MR. Histopathology demonstrated dense fibrous tissue, aggregates of histiocytes with round to oval vesicular nuclei, and other features diagnostic of Rosai-Dorfman disease; a right nasal biopsy prior to surgery showed similar microscopic findings.
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PMID:Sinus histiocytosis with massive lymphadenopathy: a case of simultaneous upper respiratory tract and CNS disease without lymphadenopathy. 842 94

Sinus histiocytosis with massive lymphadenopathy is usually a benign disorder with spontaneous remission, although a protracted clinical course with death has been reported rarely. The author reports a case diagnosed by fine needle aspiration cytology, the clinical utilization of which is discussed.
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PMID:Sinus histiocytosis with massive lymphadenopathy. Report of a case with fine needle aspiration cytology. 846 39

Sinus histiocytosis with massive lymphadenopathy (SHML/Rosai-Dorfman disease) has, on rare occasions been identified as an isolated phenomenon in lymph nodes affected by malignant lymphomas. The Registry includes four cases of SHML in patients with non-Hodgkin's lymphomas and one with multiple myeloma. SHML has more recently been recorded as a focal finding in lymph nodes involved by Hodgkin's disease of the mixed cellularity type. We report two patients presenting with lymphadenopathy caused by involvement by nodular lymphocyte predominant Hodgkin's disease with focal changes of SHML, an association not previously recorded in the literature. Responsiveness of the histiocytic cells of SHML to B-cell derived cytokines is postulated as a mechanism for this phenomenon, an hypothesis previously raised in regard to the association of focal Langerhans cell histiocytosis with Hodgkin's disease and with non-Hodgkin's lymphomas.
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PMID:Focal changes of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) associated with nodular lymphocyte predominant Hodgkin's disease. 852 13

Sinus histiocytosis with massive lymphadenopathy (SHML), or Rosai-Dorfman disease, is rare histiocytic disorder of known origin which shares several cell markers with Langerhans' cell histiocytosis (LCH). Although Rosai-Dorfman cells exhibit an aberrant immunophenotype, the indolent clinical course of SHML suggests a reactive disorder rather than a neoplastic process. Until recently this was prevailing opinion concerning LCH also, but recent studies have detected clonal histiocytes in all forms of this latter condition, which is therefore considered a clonal neoplastic disorder with highly variable biological behaviour. To determine whether the histiocytic proliferation in SHML is polyclonal or clonal we used X-linked polymorphic loci to assess clonality in lesional tissues in two women. Polymorphic regions of the human androgen receptor (HUMARA) locus were amplified by polymerase chain reaction (PCR) analysis. The HUMARA locus was informative in both cases and, following digestion with methylation-sensitive enzymes, typical polyclonal X-inactivation patterns were observed. Since abnormal cells accounted for > 90% lesional tissue cells, we conclude that Rosai-Dorfman histiocytic proliferation was polyclonal in the women studied.
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PMID:Evidence for a polyclonal nature of the cell infiltrate in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). 854 85


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