Clinical features and outcomes in patients with human immunodeficiency virus-negative, Castleman disease: a single medical center study in Tunisia

Introduction: Castleman disease (CD), known as angiofollicular lymph node hyperplasia, is an uncommon condition. The two most common histological subtypes are hyaline vascular and plasma cell. We performed a retrospective analysis to define the clinic-pathological features and survival of CD, which is quite rare focusing on the particularities of our series with a review of the recent literature.Methods: This is a retrospective study conducted in the department of internal medicine of Hedi Chaker hospital in Sfax, Tunisia over 25 years. The disease was histologically confirmed in all patients. For each file, we collected a set of data by filling in a pre-designed form.Results: 18 patients were included. There were 8 men and 10 women with a mean age of 42.8 years. CD was monocentric in 5 cases (28%) and multicentric in 13cases (72%). Clinically, peripheral adenopathy was present in 77.7% of patients and deep adenopathy in 72.2%. Systemic signs were found in 13patients, including general condition (4.4%), fever (16.6%), serositis (27.7%), and skin involvement (33.3%).A biological inflammatory syndrome accompanied the clinical picture in 66% of patients. Abnormalities in the blood count were found in 12cases (66%), with anemia in 11cases, thrombocytosis in 3cases, and hypereosinophilia in 3cases.Cutaneous Kaposi's sarcoma was associated with Castleman's disease in 2cases, Hodgkin's lymphoma, angioimmunoblastic T-cell lymphoma, and lymph node T-cell lymphoma were found in 1case respectively. 3 of the patients had associated connective tissue diseases such as Sjogren's syndrome in 2 cases and rheumatoid arthritis in 1case. HHV8 serology was positive in 1 case with a multicentric plasma cell form.Histologically, the plasma cell form represented 50% of cases, hyaline-vascular (39% of cases), and mixed (11% of cases).Therapeutically, high-dose corticosteroid therapy was initiated in 13 cases. As a second-line treatment, MOPP chemotherapy was used in 1case due to transformation into Hodgkin's lymphoma, and biotherapy (rituximab) was used in 2cases in the multicentric form. Surgical removal of superficial adenopathy was performed in 2patients with monocentric CD.Conclusion : Castleman's disease (CD) is a non-malignant lymphoproliferation of localized or multicentric form with a wide and heterogeneous clinical spectrum. Diagnosis can be difficult due to the lack of clinical and radiological specificity. Management depends on the clinical form involving surgical and/or medical management.