Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
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), 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.
 ...
PMID:Parvovirus B19 detected in Rosai-Dorfman disease in nodal and extranodal manifestations. 1714 76

Lymphadenomegaly is a common sign of benign and malignant disorders. In our practice, only every fifth patient is found to have primary or secondary nodal malignancy. Benign disorders, including banal infections and other non-neoplastic conditions, however, cause most of the cases. Among these, there are some rare entities, resulting in persistent lymphadenopathy that may cause differential diagnostic problems in the daily practice. We report here three patients, having Rosai-Dorfman disease, multicentric Castleman's disease and Kikuchi's lymphadenitis, who exemplify such cases. Our purpose with this presentation is to emphasise importance of vigorous co-operation between clinicians and pathologists. In general, it is imperative to prefer specialised haematopathological laboratories that may facilitate proper diagnosis.
 ...
PMID:Uncommon lymphadenopathies of immunopathogenesis can be misinterpreted as malignant diseases. 1716 Jun 84

Sinus histiocytosis with massive lymphadenopathy (SHML, Rosai-Dorfman disease), is a well-defined histioproliferative disorder. The extranodal type of the disease is not uncommon, but CNS involvement is extraordinary. Moreover, the isolated extranodal intracranial manifestation of the disease is extremely rare. Only a few cases of the latter form have been reported. We present a case of meningioma-like intracranial lesion, which after total resection, pathologically and immunohistochemically proved to be an isolated form of the Rosai-Dorfman disease. No concurrent nodal involvement was found, even after a detailed postoperative diagnostic work-up.Two years follow-up disclosed full recovery from the CNS symptoms, while no other SHML signs were noted. The extranodal form of the Rosai-Dorfman Disease, nowadays has been enlisted in the differential diagnosis of the space occupying intracranial and skull base lesions. However, a number of questions, dealing with the preoperative diagnosis, the surgical strategies, as well as the natural history, the prognosis of the disease, and the possible need for adjunctive therapy, remain obscured. Additional reports are useful to better understand this rare type of disease and may lead to the establishment of a more profitable management.
 ...
PMID:A new clinical entity mimicking meningioma diagnosed pathologically as rosai-dorfman disease. 1717 Oct 57

Sinus histiocytosis with massive lymphadenopathy (SHML) also called as Rosai Dorfman disease is a rare histiocytic proliferative disorder of unknown etiology. Histological features currently define it. Persistent painless lymphadenopathy due to expansion of sinuses infiltrated with benign histiocytes and plasma cells and emperipolesis are the characteristic features of SHML. Our study includes seven cases (5 nodal and 2 extranodal) of SHML over a 5-year period whose slides and blocks were reviewed. IHC was performed on the main lesion, from a panel of S100, CD68, LCA, CD20, CD3, CD30, CD43, bcl2, cytokeratin and epithelial membrane antigen. In our series we have work up available in 7 cases out of which a detailed follow-up is available in 5 patients. Out of these 5 patients, 4 have a stable disease, while one developed histiocytic sarcoma after a gap of four years.
 ...
PMID:Sinus histiocytosis with massive lymphadenopathy--a review of seven cases. 1718 39

Rosai-Dorfman disease (RDD) involves the gastrointestinal tract only in exceptional cases, and this very unusual site of presentation can confuse the pathologist. We present a case of RDD manifesting as an intestinal occlusion caused by colonic diverticulitis. The patient was a 79-year-old man with myelodysplasia, who presented with fever, abdominal pain, and constipation. Colonoscopy revealed sigmoiditis and diverticulosis. Microscopic study of the sigmoid colon surgical specimen showed the histological and immunological features of RDD. No human DNA of herpesvirus types 6 and 8 (HHV6/HHV8), Epstein-Barr virus (EBV), and cytomegalovirus (CMV) was detected in tissue by polymerase chain reaction. Electron microscopic study revealed no microbes or viral particles. Widespread nodal and extranodal RDD occurred, and the patient died 2y after initial surgery. The etiology of RDD is still under debate. We discuss the association of RDD with hematological disorders.
 ...
PMID:Intestinal occlusion caused by Rosai-Dorfman disease mimicking colonic diverticulitis. 1740 Mar 99

Sinus histiocytosis with massive lymphadenopathy (SHML) is a benign proliferating histiocytic disorder, predominantly of lymph nodes with extra-nodal involvement in some cases. It is a self-limiting disease and has a good prognosis; however some patients need steroid therapy. Therein lays a need to differentiate it from other lympho proliferative disorders of poorer prognosis. Clinically, it is suspected only when there is a significant lymph node enlargement in younger age group as the term signifies. Recently, we came across a case where a middle-aged female presented with a subtle swelling in submental region, clinically suspected of reactive or tubercular etiology. However on fine needle aspiration cytology, the smears showed many histiocytes, some of them showing lymphocytes and plasma cells in their cytoplasm, a morphological feature called as "emperipolesis" classically seen in SHML. The present case is reported because of unusual clinical presentation and possibly the need for a revision of existing terminology.
 ...
PMID:Sinus histiocytosis with massive lymphadenopathy. Is the lymph node enlargement always massive? 1746 13

Rosai-Dorfman disease (RDD) with thyroid involvement is extremely rare; we herein present the sixth known case with a review of the previously reported five cases and discuss the clinicopathological characteristics of this entity. RDD with thyroid involvement has occurred only in females with a mean age of 56.3 years, which is significantly different from nodal RDD predominantly seen in young males. Besides this, autoimmune thyroiditis is frequently associated in RDD with thyroid involvement, which raises a suspicion of a common pathogenesis of both entities. RDD with thyroid involvement is commonly misdiagnosed preoperatively as thyroid malignancy with lymph node metastasis, which may lead to overtreatment and postoperative complication. Clinical presentations might be helpful for a preoperative differential diagnosis; while thyroid function, sonography, thyroid isotope scan, and fine needle aspiration have been less useful. All six cases were treated by surgery and complete remission was seen. However, two patients with a preoperative diagnosis of benign disease suffered from hypothyroidism due to total thyroidectomy.
 ...
PMID:Thyroid involvement in Rosai-Dorfman disease. 1754 78

Originally described as sinus histiocytosis with massive lymphadenopathy, Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. A rare entity, RDD is often under-diagnosed because of a low index of suspicion by both radiologist and pathologist. Through this article, we wish to apprise radiologists of the spectrum of disease that can be encountered in this disorder. RDD can mimic other common childhood skeletal diseases including benign Langerhans cell histiocytosis and lymphoma. The clinical and radiological manifestations of RDD vary depending upon organ involvement, and its imaging features are often confused with those of other disorders. RDD should be considered in the differential diagnosis of unifocal and multifocal skeletal involvement caused by granulomatous diseases, infections, pseudogranulomatous lesions and malignancy. As long-term outcome is usually good, a conservative approach is justified in most cases. Contrasted with its typical appearance, presenting with bilateral symmetrical cervical adenopathy (as shown in one patient), we also report extranodal involvement of bone in two patients. Extranodal disease occurs along with concomitant nodal disease in about 43% of patients. In 23% of patients, isolated extranodal RDD can be seen, most commonly in the head and neck. In two of our patients, we observed extranodal involvement with skeletal involvement away from the head and neck not associated with lymphadenopathy. Skeletal involvement in RDD without lymphadenopathy is rare, occurring only in 2% of all the patients reported to date.
 ...
PMID:Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): a clinicoradiological profile of three cases including two with skeletal disease. 1824 43

Rosai-Dorfman disease (RDD), otherwise known as sinus histiocytosis with massive lymphadenopathy, is an uncommon, benign systemic histio-proliferative disease characterised by massive lymphadenopathy particularly in the head and neck region, and is often associated with extra-nodal involvement. We report a case in which RDD was mistaken for a chronic subdural hematoma and surgery was attempted.
 ...
PMID:Rosai-Dorfman disease mimicking chronic subdural hematoma. 1882 56

Rosai-Dorfman disease (RDD) is a rare benign proliferative disorder of histiocytes in the lymph nodes with or without extranodal involvement. RDD limited to the skin without nodal involvement, known as cutaneous Rosai-Dorfman disease, is very rare. We describe a 34-year-old female with RDD of the skin over the chest. A large nodule with satellite lesions was excised for histopathological examination. Microscopically, there were many large histiocytes (Rosai-Dorfman cells) exhibiting emperipolesis, among many plasma cells, lymphocytes and neutrophils throughout the dermis and the subcutaneous tissue. The histiocytes were immunohistochemically positive for S-100 protein but negative for CD 1a. Physical examination showed no lymphadenopathy or any extra-cutaneous lesions. Serological tests indicated a past infection with Epstein-Barr virus and cytomegalovirus. The diagnosis of cutaneous RDD may be difficult in the absence of associated lymphadenopathy or any specific features of the skin lesion. Hence, not only is histopathological examination required for definitive diagnosis but a high index of suspicion by the clinicians and pathologists is essential to help diagnose this very rare disease.
 ...
PMID:Cutaneous Rosai-Dorfman disease. 1910 29


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>