Rosai-Dorfman Disease with Brain Damage in One Case at the National Hospital of Zinder

Introduction: Rosai-Dorfman-Destombes disease (MRDD), or non-Langerhansian histiocytosis, is characterized by the presence of multiple polyadenopathies. It is a rare myeloproliferative disorder of histiocytes with a broad spectrum of clinical manifestations and distinctive morphological features, with abnormal accumulation of histiocytes (in emperipolesis) in lymph nodes. Observation: A 16-year-old Black male, orphaned by his father and his mother, a housewife, from a sibling group of six (06) children, of which he is the eldest, with no particular pathological history, was admitted to our clinical haematology department at the Zinder/Niger National Hospital for chronic painless bilateral cervical polyadenopathy associated with persistent fever resistant to the usual analgesics. The diagnosis of lymph node tuberculosis was considered. The patient received standard anti-tuberculosis treatment with good compliance for six (06) months. The course was marked by persistent night sweats and fever, which remained at a plateau despite antipyretics. Repeated transfusions were observed. Conclusion: Rosai-Dorfman-Destombes disease (RDDD) is a histioproliferative disorder characterized by lymphadenopathy. It is a type of histiocytosis that usually appears in children as bilateral cervical lymphadenopathy. It can manifest itself in different ways, depending on the location of the lesion. It remains a major diagnostic challenge for clinicians. Cases involving the central nervous system are rare.