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Background: Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare non-malignant disorder characterized by excessive proliferation of histiocytes, the cause of which remains unknown. Although the lymph nodes are the most commonly affected site, some patients may present with extranodal involvement, particularly in the skin, nasal cavity, eyes, and bones. In this report, we aim to present a unique case of RDD with pleural involvement in a 61-year-old patient.

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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.

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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.

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