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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 is a benign lymphoproliferative disorder growing in frequency as awareness of the disease increases. Also known as Rosai-Dorfman disease, it typically presents as painless cervical lymphadenopathy with fever and malaise. A review of the literature reveals approximately 400 cases of extranodal involvement and approximately 44 cases of central nervous system involvement. Less than 10 of the reported central nervous system tumors have presented as an epidural spinal cord tumor. The authors describe the case of a 29-year-old woman with progressive paraplegia and leg pain. Magnetic resonance imaging of the thoracic spine revealed a tumor mass from T5 to T9. The tumor was resected, and the spinal column was stabilized with pedicle screw fixation and fusion. Postoperative treatment was initiated with radiation and physical rehabilitation. The following is a case report with literature review of the entity.
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PMID:Atypical presentation of sinus histiocytosis with massive lymphadenopathy as an epidural spinal cord tumor: a case presentation and literature review. 1580 Apr 41

Rosai-Dorfman disease is a rare non-neoplastic lymphoproliferative condition. It commonly affects individuals between third and fifth decades, most common presentation being in the form of massive painless cervical lymphadenopathy with fever, weight loss and malaise. Isolated intracranial involvement is rare seen in less than 5% of patients with extranodal involvement. We present a patient with isolated contiguous cranio-spinal involvement who presented to us with remitting symptoms.
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PMID:Isolated cranio-spinal involvement of Rosai-Dorfman disease: case report. 2082 89

A case of cutaneous Rosai-Dorfman disease (CRDD) presenting as a granulomatous rosacea-like rashs was reported. A 45-year-old Chinese woman presented with a 1-month history of a widespread nonpruiginous papulonodular eruption. The rash had begun on her face and rapidly progressed to involve the neck and extremities. She was otherwise healthy, with no history of fever, malaise, or weight loss. Physical examination revealed multiple symmetrically distributed discrete and coalescing red plaques, papules and nodules scattered over the face, neck and extremities. No appreciable lymphadenopathy or hepatosplenomegaly was noted. There was no mucosal involvement. The biopsy specimen obtained from the face demonstrated the epidermis was normal, while the superficial dermis contained sheets of histiocytes with abundant, focally foamy cytoplasm. The histiocytes were surrounded by a patchy lymphocytic and plasma cell infiltrate. There was no significant histiocytic atypia. Some of these histiocytes engulfed, without destroying, lymphocytes and neutrophils (emperipolesis). Immunohistochemical staining revealed that the histiocytes were strongly positive for S100 protein, weakly positive for CD68, and negative for CD1a. A diagnosis of CRDD was made. Oral prednisone therapy was initiated at a dosage of 30 mg/d for 3 weeks and then tapered over the ensuing 2 weeks. After 5 weeks of treatment, the lesions had markedly improved.
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PMID:Cutaneous Rosai-Dorfman disease presenting as a granulomatous rosacea-like rashs. 2151 80

Rosai-Dorfman disease is a rare benign histiocytic disorder that arises predominantly in lymph nodes with fever and malaise, but can affect various organs, with or without lymphadenopathy. Solitary extranodal skeletal lesions are extremely rare. Herein, we describe a case of isolated disease of thoracic spine, with vertebral body fracture. A 14-year-old girl presented with cervical back pain for about one month as the only symptom. There was no lymphadenopathy. Results of laboratory tests were within reference ranges. The radiological examinations revealed collapse, osteolytic lesion and compressive fracture, which reduced vertical diameter of C4 vertebral body. The patient made a complete recovery after a limited C4 laminectomy, spondylodesis C3/C5 vertebra and biopsy of the lesion. Histologically, nonspecific cellular milieu and atypical histiocytic proliferation with emperipolesis, with CD68 and S100 positivity, confirmed a diagnosis of Rosai-Dorfman disease. Isolated extranodal skeletal Rosai-Dorfman disease should be considered among the differential diagnoses of similar changes, such as Langerhans cell histiocytosis, Erdheim-Cheister disease, eosinophilic granuloma, osteomyelitis, metastasis and lymphoma.
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PMID:Rosai-Dorfman disease of vertebra: Case report and literature review. 2862 Nov 4

Rosai-Dorfman disease (RDD) or Sinus histiocytosis with massive lymphadenopathy is a rare and benign histiocytic proliferative disorder first described by Juan Rosai and Ronald Dorfman in 1969, whose etiology remains unknown. Since then, many cases were reported in the literature. The disease primarily involves the lymph nodes, and is characterized by painless, bilateral cervical lymphadenopathy accompanied by fever, night sweats, malaise and weight loss, reason why many patients are clinically misdiagnosed as malignant lymphoma. In some cases, extranodal involvement may be present. Leukocytosis, elevated erythrocyte sedimentation rate, and hypergamaglobulinemia are often present. The authors report a case of a 52-year-old female patient admitted to the hospital with the diagnosis of pneumonia and progressed to multiple organs failure and death. During the hospitalization an attempt to diagnose a lymphoproliferative disease trough an axillary lymph node biopsy was disappointing. The autopsy was crucial for the diagnosis, illustrating a severe and unusual presentation of Rosai-Dorfman disease.
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PMID:Rosai - Dorfman disease: a rare entity diagnosed at autopsy. 3152 94