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Achievement of Clinical Remission in Life-Threatening Rosai-Dorfman Disease With Cladribine.
Cureus
November 2024
Hematology and Medical Oncology, Kettering Health, Kettering, USA.
Rosai-Dorfman disease (RDD) is a rare proliferative histiocytic disorder characterized by sinus histiocytosis with massive lymphadenopathy, rarely presenting with severe and life-threatening extra-nodal features. The rarity of RDD, clinically variant phenotype, limited data, and lack of a current standardized management approach make treatment decisions difficult. Herein, we present a case of life-threatening, disseminated RDD with rare clinical features of recurrent pericardial effusion, bilateral pleural effusions, and abdominal tissue fibrosis successfully treated with six cycles of cladribine, achieving clinical remission.
View Article and Find Full Text PDFCase report: Pediatric intraventricular Rosai-Dorfman disease: clinical insights and surgical strategies in a decade-long observational study and literature review.
Front Oncol
November 2024
Department of Neurosurgery, The Second Affiliated Hospital of Hainan Medical University, Haikou, Hainan, China.
Background: Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy (SHML), is a rare benign disorder characterized by the proliferation of histiocytes of uncertain origin. Central nervous system (CNS) involvement, particularly intraventricular, is exceptionally rare and poses significant diagnostic challenges due to its non-specific clinical and radiographic presentation. This study aims to present a case of intraventricular RDD and review existing literature on its clinical features, treatment strategies, and prognosis.
View Article and Find Full Text PDFSinus Histiocytosis: Diagnosis by Cryo-EBUS.
Arch Bronconeumol
December 2024
Pneumology Department, Hospital Río Hortega, Valladolid, Spain.
Cytology of Bilateral Lymph Nodal Masses in a Young Adult.
Cytopathology
December 2024
Department of General Medicine, Government Medical College and Hospital-32, Chandigarh, India.
Rosai Dorfman Destombes disease, also known as sinus histiocytosis with massive lymphadenopathy, is a rare non-neoplastic idiopathic disorder characterised by histiocytic proliferation with evidence of emperipolesis. They present with a varied spectrum of clinical manifestations and cytomorphological features, posing diagnostic challenges and therapeutic dilemmas.
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