Rosai-Dorfman Disease: A Clinico-pathological Presentation of Three Cases and Review of Literature
Journal Title: Journal of Clinical and Diagnostic Research - Year 2018, Vol 12, Issue 9
Abstract
ABSTRACT Rosai-Dorfman Disease (RDD) or Sinus Histiocytosis with Massive Lymphadenopathy (SHML) is a rare histiocytic disorder of unknown aetiology which presents as a benign, self-limiting disease of phagocytic histiocytes occurring mainly in the first and second decades of life. A good knowledge of the clinicopathological features and a high index of suspicion are required for prompt and accurate diagnosis of RDD, which commonly masquerades as lymphoid malignancy. We report the clinicopathological features of three patients with RDD, aged five years, 14 years and seven years, who presented with progressive painless neck swellings. The diagnosis was made by histopathology and treatment with oral prednisolone tabs produced varied outcomes over a therapy period of two years, 1 1/2 years and one year respectively. Relapse was observed in first case, full recovery in second case and partial recovery in third case, who was lost to follow-up one year later.
Authors and Affiliations
Francis Ikechukwu Ukekwe, Uchechukwu Obiora Ezomike, Osita Uchenna Ezenwosu, Christopher Bismarck Eke, Chibueze Onyekachi Nwokoro
Keywords
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- EP ID EP524432
- DOI 10.7860/JCDR/2018/37302.12106
- Views 113
- Downloads 0
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