Cathepsin D and E co-expression in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and Langerhans' cell histiocytosis: further evidences of a phenotypic overlap between these histiocytic disorders

1994 ◽  
Vol 424 (6) ◽  
Author(s):  
M. Paulli ◽  
E. Boveri ◽  
R. Rosso ◽  
U. Magrini ◽  
E. Solcia ◽  
...  
Keyword(s):  
Cathepsin D ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Sinus Histiocytosis ◽  
Massive Lymphadenopathy ◽  
Rosai Dorfman Disease

scholarly journals Rosai-Dorfman Disease: Rare Pulmonary Involvement Mimicking Pulmonary Langerhans Cell Histiocytosis and Review of the Literature

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Rashid AL Umairi ◽  
Danielle Blunt ◽  
Wedad Hana ◽  
Matthew Cheung ◽  
Anastasia Oikonomou
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Corticosteroid Treatment ◽  
Pulmonary Involvement ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Sinus Histiocytosis ◽  
Review Of The Literature ◽  
Massive Lymphadenopathy ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Rosai Dorfman Disease

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare nonmalignant lymphohistiocytic proliferative disorder. We report a patient with RDD who presented with multiple skin lesions, pulmonary involvement, and CT manifestations mimicking Langerhans cell histiocytosis, which improved after initiation of corticosteroid treatment.

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.

Childhood Histiocytoses

2020 ◽  
pp. 162-168
Author(s):  
Maurizio Aricò ◽  
Cor van den Bos ◽  
Sheila Weitzman
Keyword(s):  
Targeted Therapy ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Clinical Spectrum ◽  
Juvenile Xanthogranuloma ◽  
Erk Pathway ◽  
Therapeutic Approaches ◽  
Activating Mutations ◽  
Rosai Dorfman Disease ◽  
Genetic Abnormalities

This chapter summarizes the clinical spectrum of the histiocytic disorders—Langerhans cell histiocytosis (LCH), haemophagocytic lymphohistiocytosis (HLH), and some uncommon histiocytic disorders, including juvenile xanthogranuloma (JXG) and Rosai–Dorfman disease—as well as the current diagnostic and therapeutic approaches in these diseases. Multiple activating mutations in the RAS–RAF–MEK–ERK pathway have recently been described in LCH. Their role in the pathophysiology of the disorder and in targeted therapy is reviewed. This chapter explains the differences between primary and secondary HLH, and reviews the genetic abnormalities playing a role in both forms of HLH.

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