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)

A case of thyroid Rosai-Dorfman disease (RDD) without apparent lymphadenopathy in a 49-year-old woman with underlying euthyroid chronic autoimmune thyroiditis, as indicated by high thyroid autoantibodies titers, is presented. The initial presentation was that of a cold, hypoechogenic nodule of left thyroid lobe which increased in size during the two years of follow up, together with new ultrasonographic findings of the right lobe. No biochemical abnormalities were found apart from mild hypercalcemia. A near total thyroidectomy was performed. Histologically, the left lobe nodule as well as the right lobe lesions consisted of typical RDD cellular population, with the pathognomonic phenomenon of emperipolesis. Infiltration to the periphery of the gland was observed and three adjacent lymph nodes were also involved. The uninvolved thyroid parenchyma showed changes compatible with chronic autoimmune thyroiditis. No other localizations or systemic manifestations of RDD were revealed. Normocalcemia was restored promptly and the patient remains free of clinically overt disease one year post-operatively.
J Endocrinol Invest 1999 Dec
PMID:Cold thyroid nodule as the sole manifestation of Rosai-Dorfman disease with mild lymphadenopathy, coexisting with chronic autoimmune thyroiditis. 1071 Feb 76

A case of subglottic narrowing caused by an extranodal deposit of sinus histiocytosis in a 29-year-old man is reported. The characteristic features of Rosai-Dorfman disease, such as massive painless cervical lymphadenopathy, fever, raised erythrocyte sedimentation rate and raised white cell count, were all found to be absent. However, typical histological features, such as diffuse lymphoplasmacytic infiltrate, Russel bodies, foamy histiocytes and histiocytes with emperipoiesis, confirmed the diagnosis. Rosai-Dorfman disease is an uncommon disorder and its presentation with progressive subglottic narrowing is extremely rare. Our experience in the management of this condition is reported and the review of literature is discussed.
J Laryngol Otol 2000 Dec
PMID:Rosai-Dorfman disease of the subglottis. 1117 71

Sinus histiocytosis with massive lymphadenopathy or Destombes-Rosai-Dorfman's syndrome is a rare benign disease of unknown etiology, usually seen in younger patients. The cases reported concerned a 15-month old Caucasian boy and an 8 year old black boy with unilateral cervical enlargement, occasional fever and without any extranodal involvement. Diagnosis was performed by superficial lymph node biopsy. No immunodeficiency was found. The patients received no therapy and a complete spontaneous resolution was seen after a few months in the two cases. The clinical presentation, histologic characteristics, pathogenesis and treatment of the Destombes-Rosai-Dorfman's syndrome are discussed.
Int J Pediatr Otorhinolaryngol 2001 Dec 01
PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): two case reports. 1170 Jan 94

Recently, a number of neoplastic and nonneoplastic entities have been reported that radiographically and clinically mimic meningiomas. Because these lesions occur infrequently and may resemble a meningioma during intraoperative analysis, they may not be considered in the differential diagnosis. This review (and case illustrations) considers some of the newly recognized and notable lesions that can mimic meningiomas, including solitary fibrous tumors, gliosarcomas, leiomyosarcomas, hemangiopericytomas, melanocytomas, Hodgkin's disease, plasmacytomas, inflammatory pseudotumors, neurosarcoidosis, plasma cell granulomas, Rosai-Dorfman disease, Castleman's disease, xanthomas, rheumatoid nodules, and tuberculomas. Awareness that these lesions involve the dura may facilitate intraoperative recognition and, in some cases, preclude unnecessary additional surgery.
Hum Pathol 2002 Dec
PMID:Dural lesions mimicking meningiomas. 1251 91

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.
Arch Dermatol Res 2003 Dec
PMID:PET imaging of Rosai-Dorfman disease: correlation with histopathology and ex-vivo beta-imaging. 1456 72

The term sinus histiocytosis with massive lymphadenopathy (SHML) was introduced by Rosai and Dorfman in 1969. Although SHML was initially described as low-pain cervical lymphadenopathy accompanied by fever, leukocytosis, an increased erythrocyte sedimentation rate, and hypergammaglobulinemia, extranodal involvement is observed in 25 to 40% of cases. This pathology is very rare and involvement of the nasal fossae and paranasal sinuses is exceptional. We present two atypical cases of extranodal involvement exclusively confined to the head and review the literature. The first case showed a dramatic involvement of facial skin and muscles, orbit, and paranasal sinuses. In the second case, the disease was limited to the nasal fossae. Both cases showed exclusive extranodal involvement limited to the head region. In the absence of lymphadenopathy, diagnosing Rosai-Dorfman disease is difficult because of the lack of the most typical manifestation of the disease.
Ear Nose Throat J 2004 Dec
PMID:Rosai-Dorfman disease with extranodal manifestation in the head. 1572 44

We present a unique case of a man presenting with an abnormal prostate and large renal mass who was ultimately found to have prostate cancer and renal Rosai-Dorfman disease. We discuss the method of diagnosis and treatment in this patient, as well as review the presentation, diagnosis, and treatment of this rare disease.
Urology 2005 Dec
PMID:Rosai-Dorfman disease presenting as extranodal renal mass. 1636 Apr 69

Sinus histiocytosis with massive lymphadenopathy (SHML), also designated as Rosai-Dorfman disease (RDD), is a rare benign reactive lymphoproliferative disorder. It is defined by a characteristic histopathology with sinus histiocytosis and haemophagocytosis known as emperipolesis. In histiocytes S100 is strongly expressed, whereas CD1a staining typically is negative. The disease mainly manifests at a single lymph node; however, multilocular and extranodal affection can occur. Causative infectious agents, and virus infections in particular, have repeatedly been suspected, although until now the origin of the disease has been unclear. Four cases of RDD (two nodal sites and two extranodal upper respiratory tract sites) were analysed for parvovirus B19 (B19) infection by immunohistochemistry to detect B19 capsid proteins VP1/VP2. In all the four cases, huge numbers of B19-positive cells were partly detected. The positive cells were identified either as lymphocytes or, in one extranodal case, also as respiratory epithelial cells. This is the first report of B19 infection in RDD tissue, indicating that B19 may be associated with the pathogenesis of SHML.
J Clin Pathol 2006 Dec
PMID:Parvovirus B19 detected in Rosai-Dorfman disease in nodal and extranodal manifestations. 1714 76

Rosai-Dorfman disease (RDD) is a rare disorder, often benign but with possible life-threatening prognosis. In most cases, specific treatment is not necessary; when required, the management of RDD is not codified to date, and various chemotherapies have been shown to be ineffective. Here, we report a patient with RDD who presented a dramatic and sustained response with cladribine. Analysis of the cytokine profile evolution shows a clear correlation between serum levels of TNF-alpha and IL-6 and disease activity. Our findings show the promising efficacy of cladribine and suggest that therapies targeting specifically cytokines might be useful in some cases of active RDD.
Haematologica 2006 Dec
PMID:Dramatic clinical efficacy of cladribine in Rosai-Dorfman disease and evolution of the cytokine profile: towards a new therapeutic approach. 1719 58

General anesthesia in patients with mediastinal masses can lead to life-threatening cardiorespiratory complications. We report the cases of 2 pediatric patients with mediastinal masses who developed serious complications during general anesthesia. The first was a 13-year-old boy with a suspected diagnosis of high-grade T-cell non-Hodgkin lymphoma, lymph node disease, and an anterior mediastinal mass. He developed negative pressure pulmonary edema secondary to severe upper airway obstruction in spontaneous ventilation. The second was a 14-year-old boy with Rosai-Dorfman disease and paratracheal lymph node involvement. He developed severe airway obstruction in the early postoperative period. The anesthetic difficulties that arise in these cases include acute airway occlusion, superior vena cava syndrome, pulmonary artery or cardiac compression, acute pulmonary edema, and cardiopulmonary collapse. The technique of choice is induction with inhaled anesthetics and maintenance of spontaneous ventilation. Neuromuscular relaxants are avoided.
Rev Esp Anestesiol Reanim 2006 Dec
PMID:[Perioperative respiratory complications in pediatric patients with mediastinal masses: a report of 2 cases]. 1730 81


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