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), or Rosai-Dorfman Disease, presents with bilateral painless cervical adenomegaly, fever, and several hematologic abnormalities. Skin is the extranodal site most frequently affected. We described four additional cases of SHML limited to the skin, emphasizing possible confusion with other dermatoses. Clinical, morphologic, and immunohistochemical aspects of four patients with cutaneous SHLM, three diagnosed in the ABC Medical Center in Mexico City and the other at the Hermanos Ameijeiras Hospital of Havana, Cuba, are reviewed. Three males and one female, 48, 35, 42, and 55 years of age, presented with chronic asymptomatic dermal nodules, papules, or plaques on trunk, extremities, and face. Skin biopsies showed dense infiltrates of foamy histiocytes, lymphocytes, and plasma cells; histiocytes presented with prominent emperipolesis and intense S100 and CD68 immunostain. The four cases reported here had histiocytic benign proliferative disorder corresponding with cutaneous SHML: Emperipolesis suggested the diagnosis. Immunohistochemistry demonstrated positivity for S100 protein in macrophages. Cutaneous lesions of SHML are easily recognized when they are found in the classical clinical picture, but as purely skin disease could be confused with other dermatoses.
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PMID:[Rosai-Dorfman disease limited to the skin. Four case reports]. 1266 3

We report the case of a woman who developed an early relapse of a squamous cell carcinoma (SCC) and was thus restaged twice within a year using [(18)F]fluorodeoxyglucose (FDG) positron emission tomography (PET). While there was no evidence of metastatic tumor outspread, focally increased FDG uptake was visible in numerous nodes but showed no change during the period between the two PET scans. These nodes, predominantly located at the proximal extremities, ranged in size from about 1 cm to over 6 cm. They were located subcutaneously, showed a red/bluish livid color and were of stout consistency. These nodes occurred first after radiochemotherapy for a non-Hodgkin's lymphoma (NHL) about 6 years earlier and slowly increased in size and number. One node of the right forearm was resected and ex-vivo beta-imaging, directly measuring the positron emission of the intranodal FDG distribution, was done and showed an overall increased glucose utilization with distinct spots of high metabolism. Histopathological work-up of the tumor showed widespread granulomatous tissue with lymphocyte follicles. Immunostaining showed the tumor to be positive for S100, CD68 and vimentin. Rosai-Dorfman disease (RDD) was diagnosed and no evidence of a potential relapse of the previous NHL was detected. RDD is a rare disease that is associated with the multifocal growth of benign tumors. The lesions are metabolically highly active. The correlation of the beta-imaging and histopathological results showed a high metabolism within granulomatous tissue with more intense metabolism within lymphocyte follicles.
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PMID:PET imaging of Rosai-Dorfman disease: correlation with histopathology and ex-vivo beta-imaging. 1456 72

We describe a case of a diffuse large B-cell lymphoma with extensive emperipolesis phenomenon. Light microscopy revealed numerous CD68-positive/S-100-negative histiocytes containing viable neoplastic hematopoietic cells in their cytoplasm. Electron microscopy confirmed these findings. In this case, there was no evidence of Rosai-Dorfman disease either clinically or histologically. This report emphasizes that emperipolesis is not restricted to Rosai-Dorfman disease and can be found in other conditions including non-Hodgkin lymphomas.
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PMID:Emperipolesis in a case of B-cell lymphoma: a rare phenomenon outside of Rosai-Dorfman disease. 1457 35

Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder with massive lymphadenopathy. We here describe RDD of a neonate who presented with paleness and hepatosplenomegaly but not lymph-node swelling. Routine laboratory studies showed anemia, thrombocytopenia, and an elevated value of gamma-glutamyl transpeptidase. Histological examination of the liver revealed a proliferation of histiocytes with abundant eosinophilic cytoplasm, which were positive for S-100 protein and CD68 but not CD1a and did not reveal Birbeck granules. Radiological studies showed hepatosplenomegaly and a narrowing of the hepatic vein, which might have contributed to hypersplenism resulting in anemia and thrombocytopenia. This case is thought to be congenital RDD without lymphadenopathy.
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PMID:Congenital Rosai-Dorfman disease without lymphadenopathy. 1469 91

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is an idiopathic condition typically associated with cervical lymphadenopathy, fever and hypergammaglobulinaemia. Extranodal involvement has been reported in diverse sites such as the skin, upper respiratory tract, orbit and the central nervous system. We document a case of intracranial RDD in a 40-year-old woman with rapid evolution over a period of three months. Clinically, the patient suffered from headache. The MRT showed a left parietal tumour with dural attachment. Histologically, the lesion consisted of pale-staining histiocytes with emperipolesis, neutrophilic granulocytes and scattered lymphocytes. Focally, the granulocytes dominated the histological picture. By immunohistochemical analysis, the characteristic histiocytes were positive for S100 protein, CD68 and FXIIIa, but negative for CD1a. No Birbeck-granula were detectable in electron microscopic analysis. Granulocytes showed a positive Anti-HHV6b immunoreaction. The tumour was diagnosed asA'an intracranial manifestation of RDD primary to the CNS with an unusual preponderance of neutrophilic granulocytes and with only scattered lymphocytes. The postoperative clinical staging showed no other manifestations of the disease. On postoperative MRI the lesion had been completely resected. No further therapy has been applied and the patient has had an unremarkable clinical course for the last ten months.
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PMID:A case of rapidly progressive Rosai-Dorfman disease restricted to the central nervous system. 1611 57

Sinus histocytosis with massive lymphadenopathy, or Rosai-Dorfman disease, is a rare histiocytic disorder that typically presents with chronic, self-limiting, cervical lymphadenopathy. We present a case, a diagnostic dilemma for multiple consultation services, of an otherwise well 17-year-old boy without lymphadenopathy who, 8 months after excision of a T9 lytic vertebral lesion and epidural mass that caused cord compression, again presented with cord compression from progressive vertebral disease, recurrent epidural mass, and development of a paraspinal mass and tibial lesion. The excised vertebral and epidural lesions, 2 paraspinal biopsies, and tibial biopsy were interpreted as chronic inflammation until large histiocytes were noted, which were positive for CD68, S100 protein, fascin, and MAC387, and demonstrated characteristic emperipolesis (lymphophagocytosis) that was diagnostic of Rosai-Dorfman disease. This atypical clinical behavior and sites of involvement of multiple bones but not of lymph nodes is unusual and constitutes the aggressive end of the clinical spectrum and a rare cause for cord compression.
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PMID:Extranodal Rosai-Dorfman disease with multifocal bone and epidural involvement causing recurrent spinal cord compression. 1621 43

Rosai-Dorfman disease (RDD) is a non-neoplastic proliferative histiocytic disorder that primarily affects lymph nodes (sinus histiocytosis with massive lymphadenopathy). Primary RDD of the central nervous system is most uncommon. We report on a 35-year-old man with isolated RDD of the meninges overlying the left cerebral hemisphere. Presenting signs and symptoms included severe progressive ipsilateral headaches of 4 months duration, as well as laboratory evidence of mild non-specific systemic inflammatory reaction. On magnetic resonance imaging, the lesion was seen as a contrast-enhancing, plaque-like thickening of the dura mater over the left convexity,without impinging on adjacent bone or cerebral parenchyma. Meningeal biopsy revealed a mixed mononuclear infiltrate dominated by CD68(+), S100(+), CD1a(-) non-Langerhans type histiocytes on a background of fibrosis. Bacteria, in particular mycobacteria, and fungi were excluded with special stains. Extensive clinical workup, encompassing computed tomography of thoracal and abdominal organs, bone marrow biopsy, and bronchoalveolar lavage failed to reveal any extracranial involvement. Laboratory tests for autoimmunity, including C- and P-antineutrophil cytoplasmic antibodies, antinuclear antibody, and serum rheumatoid factor, were negative. Methylprednisolone therapy induced complete remission of symptoms, with the neuroradiologic status remaining unchanged on follow-up after 2 months. We discuss the complex clinicopathologic differential diagnosis and therapeutic issues of this rare condition. While the correct diagnosis of central nervous system RDD is unlikely to be established without invasive procedures (biopsy), a conservative therapeutic approach may be considered a legitimate option.
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PMID:Isolated Rosai-Dorfman disease of intracranial meninges. 1637

A 3-year-old Thai boy suffered from two histiocytoses, Rosai-Dorfman disease (RDD) and juvenile xanthogranuloma (JXG). The patient first presented with massive cervical lymphadenopathy at the age of one year. Biopsy revealed typical RDD; abnormally large CD68- and S-100 protein-positive histiocytes with occasional emperipolesis filled up the sinuses. Two years later, he developed polyuria and polydypsia. Skull film demonstrated osteolytic lesions at the occiput and left parietal region. Enlargement of the pituitary stalk was found on the magnetic resonance imaging. Despite the clinical impression of Langerhans cell histiocytosis, biopsy of the occipital lesion disclosed numerous large histiocytes with foamy cytoplasm. Several Touton giant cells with wreath-like arrangement of the nuclei were also observed. The abnormal cells expressed CD68 and factor XIIIa, but were non-reactive with S-100 protein and CD1a. Biopsy of the pituitary stalk was not performed According to the authors' literature search, this represents the first report of RDD and JXG affecting the same person.
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PMID:Rosai-Dorfman disease and juvenile xanthogranuloma in a Thai boy: report of a case. 1658 88

Reticulohistiocytoma and multicentric reticulohistiocytosis are designations for uncommon, incompletely characterized histiocytic proliferations of the skin or soft tissues. In this study, we analyzed a uniform group of 44 lesions composed of epithelioid histiocytes, comprising a subset of lesions originally designated as reticulohistiocytoma, and propose designating them as "solitary epithelioid histiocytoma" (SEH), in line with the recently published classification proposal for histiocytic disorders. There were 26 males and 18 females with a median age of 35 years (range, 2.5-74 years). All patients had a superficial, circumscribed, mildly elevated, solitary lesion (size range, 1.5-11 mm; median, 4 mm), located in the trunk wall (n = 16), lower extremity (n = 12), head and neck (n = 8, including 2 in the oral cavity), upper extremity (n = 6), penis (n = 1), and an unspecified site (n = 1). Histologically, the lesions typically involved upper and mid-dermis and were not ulcerated. They were composed of large epithelioid histiocytes with a varying number of lymphocytes and neutrophils. The histiocytes had abundant, typically densely eosinophilic, cytoplasm and mostly mild, if any, nuclear atypia. Multinucleated forms with randomly oriented nuclei were also present. The histiocytes had low mitotic activity (range, 0-4 mitoses per 10 wide HPFs; median, 1 mitosis per 10 HPFs). The lesions contained varying numbers of CD3-positive T cells, whereas B lymphocytes, plasma cells, eosinophils, and mast cells were scant, if present at all. Immunohistochemically, the epithelioid histiocytes were positive for CD163, CD68, lysozyme (variably), and vimentin. They often had focal nuclear immunoreactivity for microphthalmia transcription factor, and they sometimes had focal reactivity for Factor XIIIa and S-100 protein. Membrane positivity for CD31, CD43, and CD45 was variable. The epithelioid histiocytes were consistently negative for CD3, CD20, CD30, HMB45, and keratins. All 12 patients with follow-up information had an uneventful clinical course with no recurrences (median, 13 years). SEH is a benign, probably reactive, histiocytic proliferation of unknown etiology. It needs to be distinguished from Rosai-Dorfman disease, juvenile xanthogranuloma, a variety of granulomatous conditions, and some malignant neoplasms, including histiocytic sarcoma, melanoma, and epithelioid sarcoma.
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PMID:Reticulohistiocytoma (solitary epithelioid histiocytoma): a clinicopathologic and immunohistochemical study of 44 cases. 1662

Sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman disease is a rare histiocytic disorder of unknown origin. Both dendritic cell and monocyte-macrophage lineage have been considered as the origin of the histiocytes that are classical of the disorder. We analyzed seven cases of Rosai-Dorfman disease to determine the immunophenotypic profile of these cells. Immunohistochemical analysis was undertaken by using: (a) fascin as a novel mature dendritic cell marker; (b) S-100 protein; (c) CD68 (both KP1 and PGM1) as macrophage related markers; (d) CD79a and (e) CD3. The Rosai-Dorfman histiocytes exhibited strong cytoplasmic staining for fascin in all the cases and demonstratedformation of a meshwork of fine dendritic processes emanating from the cell cytoplasm. S-100 protein was uniformly expressed in all the cases. Expression of both KP1 and PGM1 epitopes of CD68 was found to be weak and variable. The phagocytosed lymphocytes were composed of an admixture of both B and T-lymphocytes. The characteristic expression pattern of the histiocytes for fascin along with co-expression of S-100 protein and a consistently weaker and variable expression of macrophage-lineage markers point more towards a dendritic cell ontogeny of these cells. Further, abundant fascin expression and presence of dendritic processes indicate a differentiated or mature dendritic cell phenotype for these cells.
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PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman disease): evidence for a dendritic cell derivation? 1676 37


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