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Published on: September 20, 2018
[Castleman disease with systemic manifestation: a case report]
Ahlam Hmimsa1, Nabil Touihem1, Hicham Attifi1
1Service d'Oto-Rhino-Laryngologie et de Chirurgie Cervico-Faciale, Hôpital Militaire Moulay Ismail, Meknès, Maroc.
None:
Castleman's disease is a rare lymphoproliferative disorder. It typically presents with lymphadenopathy and may be associated with sometimes severe systemic manifestations. It can occur in two clinical forms: the unicentric form, which is limited to a single lymph node or lymph node region, generally has a favorable prognosis and is primarily treated with surgical excision; and the multicentric form, which is more severe, characterized by diffuse lymph node involvement and excessive cytokine production, particularly interleukin-6 (IL-6), which accounts for the systemic symptoms. Treatment in the multicentric form includes monoclonal antibodies targeting IL-6, immunosuppressants, chemotherapy, or corticosteroids, depending on the underlying cause. The disease may be associated with human herpes virus 8 (HHV-8) infection, especially in immunocompromised patients. We report the case of a 65-year-old patient presenting with polyadenopathy and Raynaud's phenomenon. Imaging revealed multiple lymphadenopathies along with hepatosplenomegaly. An exploratory cervicotomy was performed, followed by histopathological examination and immunohistochemical analysis, which confirmed the diagnosis of multicentric Castleman disease. This clinical case underscores the importance of considering Castleman disease in the differential diagnosis of unexplained polyadenopathies, particularly when accompanied by atypical systemic manifestations.
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