Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a rare non-neoplastic pathologic condition that frequently pursues a prolonged clinical course marked by exacerbations and remissions. Cutaneous RDD is even less common than cases involving lymph nodes. We present the case of a patient with long-standing Crohn's disease who developed cutaneous RDD in the forearm.
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Achievement of Clinical Remission in Life-Threatening Rosai-Dorfman Disease With Cladribine.
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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 PDFRosai-Dorfman disease: A rare presentation as an isolated axillary lymphadenopathy, a case report and literature review.
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Department of Surgical Oncology, Regional Hospital of Jendouba, Jendouba, Tunisia.
Introduction: Rosai-Dorfman disease (RDD) is a rare histiocytic neoplasm. It most commonly presents with bilateral cervical lymphadenopathy. We report the fourth case in the literature of RDD presenting as isolated axillary lymphadenopathy.
View Article and Find Full Text PDFResponse to the commentary on "Cutaneous rosai-dorfman disease: a systematic review and reappraisal of its treatment and prognosis".
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Approach to the Patient: From Endocrinopathy to the Diagnosis of a Histiocytic Disorder.
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Endocrinopathies are frequently the initial presentation of histiocytic neoplasms, which are rare hematologic disorders affecting multiple organ systems. Langerhans cell histiocytosis and Erdheim-Chester disease are 2 such disorders known to infiltrate the hypothalamus and/or pituitary gland, leading to arginine vasopressin deficiency (AVP-D) and anterior pituitary dysfunction (APD) in 20% to 30% of cases, often as the first manifestation. Conversely, histiocytic disorders account for a notable proportion (10-15%) of all pituitary stalk lesions.
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