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- Title
Rosai-Dorfman Disease: A Clinico-Pathological Presentation of Three Cases and Review of Literature.
- Authors
UKEKWE, FRANCIS IKECHUKWU; EZOMIKE, UCHECHUKWU OBIORA; EZENWOSU, OSITA UCHENNA; EKE, CHRISTOPHER BISMARCK; NWOKORO, CHIBUEZE ONYEKACHI
- 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.
- Publication
Journal of Clinical & Diagnostic Research, 2018, Vol 12, Issue 9, p4
- ISSN
0973-709X
- Publication type
Academic Journal
- DOI
10.7860/JCDR/2018/37302.12106