Clinical and pathological aspects of patients with Castleman’s disease in three large centers in the state of São Paulo Thesis by Julia Mota Leite. Advisor: Carlos Sérgio Chiattone

Castleman’s Disease (CD) is a rare and heterogeneous benign lymphoproliferative disorder, with a slight female predominance among patients with unicentric Castleman’s disease (UCD) and a slight male predominance among patients with multicentric Castleman’s Disease (MCD) and the age distribution is bimodal. The pathophysiology is yet unknown. The diagnosis is reached through clinical, laboratory and pathological examination, including HHV-8. This exam might distinguish three histological types: hyaline-vascular (HV), plasma cells variant (PCV) and mixed type (MT). The treatment of CD will depend on its clinical behavior, which can be localized (unicentric) or generalized (multicentric). For the localized disease, surgical extraction is the chosen treatment. In multicentric disease, treatment with monoclonal antibodies and chemotherapy are indicated on many occasions. Purpose: To define clinical and pathological profiles of patients diagnosed with CD in three major centers in the state of São Paulo and compare the data found with those in the literature. Case Study and Methods: A retrospective study was carried out through information contained in the medical records of 51 patients, between July 1999 and June 2020. Seven patients were excluded, and 44 were analyzed in total. Results: The average age of UCD patients was 35 years-old and of MCD patients was 49 years-old (p=0,013). Regarding gender, there was a predominance of females among patients with UCD (68,4%) and males in patients with MCD (57,9%) (p=0,103). The most common site of involvement in UCD was the cervical region (36,8%). 73,7% of patients with UCD and 68,4% of patients with MCD presented the histological form HV (p=0,499). Most patients with laboratory abnormalyties had MCD. 78% of the patients were asymptomatic, with the majority of symptomatic patients with MCD (p=0,042). Only two of the 27 patients evaluated for the presence of human immunodeficiency virus (HIV) had positive serology. HHV-8 was evaluated in 14 cases, being positive in two. Of the patients with UCD, 94,7% underwent excisional biopsy, against only 41,2% of patients with MCD (p=0,01). The mean follow-up was 61 months. Conclusion: We observed similarities in the clinical profile between patients in our study and patients described in the literature, such as gender, mean age, B symptoms, visceromegaly, fluid accumulation and treatment. Unlike the literature, the cervical region was the most affected site, besides the greater association of the HV histological subtype among patients with MCD.

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