scholarly journals Rosai-Dorfman Disease

2014 ◽  
Vol 6 (3) ◽  
pp. 121-122
Author(s):  
Sushant Joshi
Keyword(s):  
Cervical Lymphadenopathy ◽  
Sinus Histiocytosis ◽  
Treatment Modalities ◽  
Unknown Etiology ◽  
Neck Swelling ◽  
Massive Lymphadenopathy ◽  
Benign Disorder ◽  
Rosai Dorfman Disease

ABSTRACT Rosai-Dorfman disease also known as sinus histiocytosis with massive lymphadenopathy is a rare benign disorder of unknown etiology, which present most commonly as bilateral massive lymphadenopathy of neck associated with fever. These cases are frequently misdiagnosed as lymphoma, and thus it is important to distinguish Rosai-Dorfman disease from other causes of neck swelling because of different treatment modalities. We report here a case of Rosai-Dorfman disease presenting with massive right cervical lymphadenopathy. How to cite this article Joshi S. Rosai-Dorfman Disease. Int J Otorhinolaryngol Clin 2014;6(3):121-122.

scholarly journals Primary Rosai-Dorfman Disease of Bone: A Report of Two Cases

2019 ◽  
Vol 2019 ◽  
pp. 1-6
Author(s):  
Andrew B. Ross ◽  
Kirkland W. Davis ◽  
Darya Buehler ◽  
Brian Y. Chan
Keyword(s):  
Young Adults ◽  
Cervical Lymphadenopathy ◽  
Sinus Histiocytosis ◽  
Osseous Lesion ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
Extranodal Disease

Rosai-Dorfman disease (RDD), sometimes known as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic disorder that most commonly presents as painless, massive cervical lymphadenopathy in young adults. Extranodal disease can occur in up to 40% of patients but primary involvement of bone is rare. We present two cases of primary RDD of bone: one case of multifocal osseous RDD presenting as a painful lesion in the elbow, and one case of a solitary osseous lesion presenting as a painful lesion in the wrist.

scholarly journals HISTOCYTOPATHOLOGICAL DIAGNOSIS OF ROSAI-DORFMAN DISEASE: CASE REPORT AND LITERATURE REVIEW

2021 ◽  
Author(s):  
Zephania Abraham ◽  
Zerd Francis ◽  
Advera Ngaiza ◽  
Aveline Kahinga ◽  
Faustine Bukanu
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Sinus Histiocytosis ◽  
Unknown Etiology ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
Disease Case

Sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease) being a rare benign proliferative self-limiting disease of the cells of macrophage-histiocyte family is of unknown etiology and presents with massive lymphadenopathy. We are hereby reporting a case of RDD presenting with massive bilateral cervical and submandibular lymphadenopathy, diagnosed by histocytopathology.

scholarly journals Rosai Dorfman Disease

2021 ◽  
Author(s):  
Kalpana Giri ◽  
Ashok Baral ◽  
Niva Tiwari ◽  
Krishna Sharma
Keyword(s):  
Rare Case ◽  
Cervical Lymphadenopathy ◽  
Unknown Origin ◽  
Sinus Histiocytosis ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
History Of

Rosai-Dorfman disease (RDD) is referred to as Sinus Histiocytosis Massive Lymphadenopathy (SHML) of unknown origin. We are reporting a rare case of Rosai Dorfman disease, a case of 6 years old male with a history of multiple painless bilateral submandibular and cervical lymphadenopathy.

Concomitant Occurrence of Sinus Histiocytosis With Massive Lymphadenopathy and Nodal Marginal Zone Lymphoma

2011 ◽  
Vol 135 (3) ◽  
pp. 390-393
Author(s):  
Changlee S Pang ◽  
David D Grier ◽  
Michael W Beaty
Keyword(s):  
Lymph Node ◽  
Marginal Zone ◽  
Lymphoproliferative Disorders ◽  
Marginal Zone Lymphoma ◽  
Sinus Histiocytosis ◽  
Unknown Etiology ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
Children And Young Adults ◽  
Nodal Marginal Zone Lymphoma

Abstract Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease, is a rare self-limiting disorder of histiocytes with unknown etiology. Sinus histiocytosis with massive lymphadenopathy is most common in children and young adults and is characterized by painless lymphadenopathy. Histologically there is a proliferation of sinus histiocytes with lymphophagocytosis or emperipolesis. On rare occasions, SHML has been associated with lymphoma, usually involving different anatomic sites and developing at different times. We report a case of concomitant SHML and nodal marginal zone lymphoma involving the same lymph node without involvement of other nodal or extranodal sites. The presence of concomitant SHML within the lymph node involved by nodal marginal zone lymphoma may represent the responsiveness of SHML histiocytes to B-cell–derived cytokines in lymphoproliferative disorders. To our knowledge, this is the first description of concomitant occurrence of SHML and nodal marginal zone lymphoma.

TNFRSF6 (Fas Antigen) Mutations in Patients with Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease).

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 2389-2389 ◽  
Author(s):  
Tracy I. George ◽  
Lisa Ma ◽  
Peter L. Nagy ◽  
Yasodha Natkunam ◽  
Roger A. Warnke ◽  
...  
Keyword(s):  
Direct Sequencing ◽  
Donor Site ◽  
Sinus Histiocytosis ◽  
Splice Donor Site ◽  
Unknown Etiology ◽  
Splice Donor ◽  
Apoptotic Pathway ◽  
Fas Antigen ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease

Abstract Sinus histiocytosis with massive lymphadenopathy (SHML or Rosai-Dorfman disease) is a rare histiocytic proliferation of unknown etiology. There is a known association between SHML and immune disorders. Recently, histologic changes of SHML have been found in lymph nodes of patients with autoimmune lymphoproliferative syndrome (ALPS), a disorder most commonly associated with mutations in the TNFRSF6 gene that encodes the Fas antigen. We retrospectively analyzed tissue from 22 patients with SHML for TNFRSF6 gene mutations using isolated genomic DNA from paraffin-embedded tissue followed by screening of PCR products for the promoter regions and all exons (1–9) of the gene for possible mutation by high performance liquid chromatography. Mutations were confirmed by direct sequencing. We identified six patients (27%) with SHML and mutations of the TNFRSF6 gene as listed below, including three cases (14%) with potentially significant mutations (*) involving promotor and splice donor regions, and frameshift mutations. The samples with mutations had a 4:2 male:female ratio with a mean/median age of 39/43 years (range 7–68 years) compared to a 9:7 male:female ratio, mean/median age of 42/43 years (range 12–76 years) for the non-mutated samples. No differences in site of disease were identified in the mutated versus non-mutated groups. None of the samples had morphologic features to suggest ALPS. These results suggest that at least a subset of cases of SHML are associated with disruption of the Fas apoptotic pathway. Additional study of other components of the apoptotic pathway may be warranted in this disease. Cases with TNFRSF6 mutations. Case No. Nucleotide Change Localization Codon Amino acid change **Numbering for the promoter sequence, according to www.mutationdiscovery.com. All other numbering according to GeneBank accession number M67454. 1 c217+34A>G Intron 7 c217+40A>G Intron 7 2 T779C Exon 7 195 Val --> Ala 3 c217+1G>A Intron 7 Splice defect* T763C Exon 7 190 Val --> Ala c217+182A>G Intron 7 c217+183A>G Intron 7 C1425T Exon 9II 3′UTR * 4 597del T Exon 4 135 Frameshift* 5 T/C 4656** Promoter * c189+4A>T Intron 6 5′ consensus splice donor site* C729T Exon 6 179 Leu --> Phe 6 c218-84A>G Intron 7

Primary Pancreatic Sinus Histiocytosis With Massive Lymphadenopathy (Rosai-Dorfman Disease): An Unusual Extranodal Manifestation Clinically Simulating Malignancy

2010 ◽  
Vol 134 (2) ◽  
pp. 276-278 ◽  
Author(s):  
Mark Podberezin ◽  
Ronald Angeles ◽  
Grace Guzman ◽  
David Peace ◽  
Sujata Gaitonde
Keyword(s):  
Adipose Tissue ◽  
Lymph Node ◽  
Unusual Case ◽  
Sinus Histiocytosis ◽  
Gastrointestinal System ◽  
Rare Entity ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease

Abstract Sinus histiocytosis with massive lymphadenopathy (SHML), also called Rosai-Dorfman disease, is a rare entity. Its etiology and pathogenesis are still essentially unclear. The histologic hallmark of this disease is proliferation of distinctive histiocytes within lymph node sinuses and in extranodal sites. Approximately 23% of patients with SHML, documented in the SHML Registry, presented with disease primarily in extranodal sites, and very few cases of SHML (<1%) involving the gastrointestinal system have been described in the literature. We report an unusual case of primary pancreatic SHML with infiltration of the process into peripancreatic, perinephric, and perisplenic adipose tissue, simulating malignancy.

Cytomorphology and Histology Correlation of Rosai-Dorfman Disease: A 15-Year Study from a Tertiary Referral Centre in South India

2016 ◽  
Vol 61 (1) ◽  
pp. 55-61 ◽  
Author(s):  
Abid Hussain ◽  
Ashwani Tandon ◽  
Aruna K. Prayaga ◽  
Tara Roshni Paul ◽  
Anukonda M.V.R. Narendra
Keyword(s):  
Hodgkin Lymphoma ◽  
Plasma Cells ◽  
Cervical Lymphadenopathy ◽  
Cost Effective ◽  
Needle Aspiration ◽  
Unknown Etiology ◽  
Tertiary Referral Centre ◽  
Reliable Technique ◽  
Male Predominance ◽  
Rosai Dorfman Disease

Objectives: Rosai-Dorfman disease (RDD) is an uncommon, benign histiocytic disorder of unknown etiology, typically presenting in young adulthood. We highlight the cytomorphology of RDD and correlate it with the histopathology. Study Design: All cases diagnosed as RDD on fine-needle aspiration cytology between January 2001 and June 2015 were included. Clinical details were obtained from medical records. The cytology smears were reviewed along with the histopathology and immunohistochemistry, wherever available. Results: The study included 10 cases ranging in age from 11 to 68 years (median 29). There was a male predominance with a male:female ratio of 1.5:1. The patients commonly presented with bilateral cervical lymphadenopathy. Extranodal involvement was seen in 2 cases in the nose and mandible, respectively. Of these 10 cases, 8 were later biopsied. The cytological features included numerous crescentic histiocytes, emperipolesis, reactive lymphocytes and plasma cells. A histological diagnosis of RDD was made in 7 out of 8 cases, and 1 was diagnosed as Hodgkin lymphoma. Conclusion: FNA represents an efficient, minimally invasive, cost-effective and reliable technique for the diagnosis of RDD and may obviate the need for further biopsy. However, the disease has close differential diagnoses, including Langerhans cell histiocytosis, granulomatous lesions, and Hodgkin lymphoma. Hence, it must be remembered that there can be pitfalls when the diagnosis is made by cytology alone.

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