Rosai-Dorfman Disease: A Clinico-pathological Presentation of Three Cases and Review of Literature
Published: September 1, 2018 | DOI: https://doi.org/10.7860/JCDR/2018/37302.12106
Francis Ikechukwu Ukekwe, Uchechukwu Obiora Ezomike, Osita Uchenna Ezenwosu, Christopher Bismarck Eke, Chibueze Onyekachi Nwokoro
1. Senior Lecturer/Consultant Pathologist, Department of Morbid Anatomy, University of Nigeria Enugu Campus, Enugu/University of Nigeria Teaching Hospital, Ituku
Ozalla, Enugu, Nigeria.
2. Senior Lecturer/Consultant Paediatric Surgeon, Sub-Department of Paediatric Surgery, University of Nigeria Enugu Campus, Enugu/University of Nigeria Teaching
Hospital, Ituku Ozalla, Enugu, Nigeria.
3. Senior Lecturer/Consultant Paediatrician, Department of Paediatrics, University of Nigeria Enugu Campus, Enugu/University of Nigeria Teaching Hospital, Ituku Ozalla,
Enugu, Nigeria.
4. Senior Lecturer/Consultant Paediatrician, Department of Paediatrics, University of Nigeria Enugu Campus, Enugu/University of Nigeria Teaching Hospital, Ituku Ozalla,
Enugu, Nigeria.
5. Lecturer II/ Senior Registrar in Pathology, Department of Morbid Anatomy, University of Nigeria Enugu Campus, Enugu/University of Nigeria Teaching Hospital, Ituku
Ozalla, Enugu, Nigeria.
Correspondence
Dr. Francis Ikechukwu Ukekwe,
Senior Lecturer/Consultant Pathologist, Department of Morbid Anatomy, University of Nigeria Enugu Campus,
Enugu/University of Nigeria Teaching Hospital, Ituku Ozalla, Enugu, Nigeria.
E-mail: drikukekwe@hotmail.com, francis.ukekwe@unn.edu.ng
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.
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