Castleman Disease in Tunisia: A Multicenter Clinical, Prognostic, and Therapeutic Study

Castleman disease is an uncommon benign lymphoproliferative condition that includes diverse clinical, biochemical, and histological variations, each characterized by unique prognoses and therapeutic approaches. The aim of our study was to characterize the clinical and paraclinical features, as well as the management approaches, of Castleman disease in the Tunisian population.

We conducted a multicenter study including patients with a histologically confirmed diagnosis of Castleman disease who were followed in the Internal Medicine departments of various hospitals across Tunisia between 2000 and 2024.

Sixteen patients (55.2%) with multicentric Castleman disease and 13 patients (44.8%) with the unicentric form were included. The most frequent initial manifestations were lymphadenopathy (69%), mass (20%), fever (26.7%), and weight loss and fatigue (56.7%). HHV-8 tested positive in 10.7%, while 3.6% of patients had autoantibodies. Hyaline-vascular (53.6%), plasmacytic (32.1%), and mixed (14.3%) subtypes were diagnosed, with a distinct plasmacytic predominance among multicentric types. Following total surgical resection, 14 patients (48.3%) experienced immediate radiological and clinical remission. On the other hand, systemic therapy was required for patients with multicentric types. In total, 12 patients (44.4%) experienced full remission, (14.8%) remained stable, and (3.7%) continued to progress despite treatment.

With a significant percentage of multicentric forms and a low HHV-8 seropositivity rate, our Tunisian study validates the various manifestations of Castleman disease.