RESUMO
Background: multicentric Castleman disease is a rare lymphoproliferative disorder of unknown origin. Castleman disease [CD] also known as angiofollicular lymph node hyperplasia, is a rare lymphoproliferative disorder with poorly understood pathophysiology. The multicentric plasma cell variant is highly associated with infection by human herpesvirus 8 [HHV8], and patients have an increased risk for the development of other HHV8-associated neoplasms, including Kaposi's sarcoma and extranodal B-cell lymphoma. The authors describe a 50-year-old woman that presented with protracted fever with diagnosis of Multicentric Castleman disease
Case: we report a 50-year-old woman that presented with a 5 months history of protracted fever, night sweating, abdominal pain, dyspnea, loss of appetite and weight loss approximately 5 kg. Physical examination was significant for a febrile [39 degree c], ill-appearing in mild distress with a distended and diffusely tender abdomen with moderate splenomegaly, pitting edema on lower limbs and disseminated lymphadenopathy. Despite a comprehensive evaluation, her diagnosis remained elusive for several weeks. Eventually, a lymph node biopsy showed the presence of Multicentric Castleman disease. Due to her relatively poor prognosis and severity of the disease, she was treated with combination chemotherapy consisting of cyclophosphamide, vincristine, and prednisone. She tolerated her therapy well and is currently free of disease at 6 months of follow-up
Conclusions: according to low prevalence of multicentric CD and loss of standard treatment for it and due to the poor prognosis in adults with multicentric CD, the potential for malignancy, we recommend further evaluations [lymph node biopsy] for diagnosis Multicenteric CD in any patients with compatible signs and symptom