scholarly journals Secondary biphenotypic leukemia following Rosai-Dorfman-Disease A coincidence?

2021 ◽  
Author(s):  
Anne Thieme ◽  
Katja Maurus ◽  
Karen Ernestus ◽  
Steffen Hirsch ◽  
Kathrin Schramm ◽  
...  
Keyword(s):  
Saliva Sample ◽  
Leukemic Cells ◽  
Treatment Resistant ◽  
Lymphoid Malignancies ◽  
Massive Lymphadenopathy ◽  
Whole Exome ◽  
Rosai Dorfman Disease ◽  
Common Marker ◽  
Cancer Predispostion ◽  
Biphenotypic Leukemia

Rosai-Dorman-Disease is one subgroup of Non-Langerhans cell histiocytosis with massive lymphadenopathy. It is a generally a benign and self-limiting disease, but an association to hemato-lymphoid malignancies or immune diseases is suspected. We present the case of a 6-year old male patient with multifocal extranodal RDD developing a secondary biphenotypic, treatment-resistant leukemia during treatment. Hypothysing that RDD and the secondary leukemia could be based on the same malign clone or on a cancer predispostion syndrome, whole-exome-sequencing of a saliva sample and leukemic cells were performed. A common marker of cell lines of histiocytosis and leukemic blasts has not been found.

Rosai–Dorfman Disease: A rare cause of massive lymphadenopathy

2019 ◽  
Vol 153 (9) ◽  
pp. e52
Author(s):  
Marta Custódio ◽  
Isa Silva ◽  
Anabela Salgueiro Marques
Keyword(s):  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease

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.

Rosai-Dorfman Disease of the Parotid Gland

2001 ◽  
Vol 125 (10) ◽  
pp. 1348-1350
Author(s):  
Ridas Juskevicius ◽  
James L. Finley
Keyword(s):  
Salivary Gland ◽  
Lupus Erythematosus ◽  
Plasma Cells ◽  
Correct Diagnosis ◽  
Unknown Origin ◽  
Needle Aspiration ◽  
Major Salivary Gland ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
Mixed Inflammatory Infiltrate

Abstract Rosai-Dorfman disease is a rare histiocytic proliferative disorder of unknown origin and a distinct clinicopathologic entity also known as sinus histiocytosis with massive lymphadenopathy. The disease can involve extranodal tissues and rarely can present as salivary gland enlargement without significant lymphadenopathy. Involvement of the extranodal head and neck sites appears to be more common in patients with immunologic abnormalities. The disease was first described in 4 patients in 1969, and with later descriptions of more patients, the disease was established as a well-defined clinicopathologic entity. The characteristic pathologic feature of this disease is proliferation of distinctive histiocytic cells that demonstrate emperipolesis in the background of a mixed inflammatory infiltrate, consisting of moderately abundant plasma cells and lymphocytes. Fine-needle aspiration biopsy can be helpful in establishing the correct diagnosis, since surgical treatment is not necessary other than obtaining tissue for definitive diagnosis. We describe cytologic, histopathologic, and immunohistochemical features of a case of Rosai-Dorfman disease that involved a major salivary gland without significant lymphadenopathy in a 48-year-old patient with systemic lupus erythematosus. We also briefly discuss possible causes and pathogenesis and review the literature.

scholarly journals Intracranial And Intraorbital Rosai Dorfman Disease

2020 ◽  
Vol 11 (4) ◽  
pp. 5187-5191
Author(s):  
Sivapriya G Nair ◽  
Jina Raj ◽  
Sajesh K Menon ◽  
Suhas Udayakumaran ◽  
Roshni P R
Keyword(s):  
Histopathological Study ◽  
Disease Treatment ◽  
Mri Scan ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease ◽  
History Of ◽  
The Central Nervous System ◽  
The Right ◽  
The Brain ◽  
Suprasellar Extension

Rosai Dorfman disease is a rare histiocytic disorder. It is also known as Sinus Histiocytosis. It is with massive lymphadenopathy involves an overproduction of a type of white blood cell. The disease is rarely associated with intracranial and intraorbital involvement. Intracranial Rosai-Dorfman can mimic meningioma. Other pathologies also underline its pathologies. Here, we report a nine-year-old boy with a history of proptosis of the right eye and presenting with multiple skull lesions. Histopathological study revealed Sphenopetroclival lesion, which features that of Rosai Dorfman Disease. His MRI scan of the brain was taken, which showed evidence of right optic nerve meningioma with sella and suprasellar extension, causing severe proptosis. The child underwent right frontotemporal craniotomy with petrosectomy and Transylvanian, subtemporal approach to multicompartmental Rosai-Dorfmans lesion. After four months, the patient had a recurrence of the disease on which chemotherapy and steroids were started, which also did not show much response while taking an MRI scan. A corticosteroid is a useful option in the Central Nervous System Rosai Dorfman disease treatment. But this patient showed a negative outcome to the treatment.

scholarly journals DIAGNOSIS OF ROSAI-DORFMAN DISEASE BY FINE NEEDLE ASPIRATION CYTOLOGY- A STUDY OF ELEVEN CASES WITH EMPHASIS ON SITE

2020 ◽  
pp. 1-3
Author(s):  
Rinsha Surendranath ◽  
M Rajani
Keyword(s):  
Lymph Node ◽  
Lymph Nodes ◽  
Cervical Lymph Node ◽  
Age Distribution ◽  
Needle Aspiration ◽  
Cervical Region ◽  
Axillary Lymph ◽  
Diagnostic Significance ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease

Rosai Dorfman Disease also called as Sinus Histiocytosis with Massive Lymphadenopathy is a benign proliferative disorder of histiocytes which typically affects lymph nodes of cervical region ,however extranodal sites are also being reported by many authors[1].FNAC is the investigation of choice as is it less invasive and more reliable.Aims :To study the various sites of involvement and diagnostic significance of FNAC along with the pattern of site and age distribution .We studied a series of 11 cases for a period of one year.Materials and methods: Eleven cases studied in the department of Pathology Govt. Siddhartha Medical College,Vijayawada.Material collected from Govt. General Hospital,Vijayawada.Results:The present study showed two extranodal,one axillary lymph node,one inguinal lymph node and seven cervical lymph node involment.Conclusion:Rosai- dorfman disease main clinical manifestion is cervical lymph node enlargement ,but other lymph nodes and extranodal site can also be involved.Most common age of presentation was between 20-40years

Rosai–Dorfman Disease: A rare cause of massive lymphadenopathy

2019 ◽  
Vol 153 (9) ◽  
pp. e52
Author(s):  
Marta Custódio ◽  
Isa Silva ◽  
Anabela Salgueiro Marques
Keyword(s):  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease
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