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Clinical and histopathology characteristics of Castleman disease: a multicenter study of 51 Brazilian patients

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Abstract

Castleman’s disease (CD) is a rare and heterogeneous lymphoproliferative disorder, with limited available clinical information in Brazil. 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. The average age of unicentric CD (UCD) patients was 35 years old and of multicentric CD (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%). A total of 73.7% of patients with UCD and 68.4% of patients with MCD presented the histological form hialyne-vascular (HV) (p = 0.499). Most patients with laboratory abnormalities had MCD. A total of 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. 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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Authors and Affiliations

  1. Santa Casa de São Paulo Medical School, Faculdade de Ciências Médicas da Santa Casa de São Paulo, São Paulo, Brazil

    Julia Mota Leite, Leonardo Sementilli, Sérgio Augusto Buzian Brasil & Carlos Sérgio Chiattone

  2. Grupo Fleury São Paulo and Adolfo Lutz Institute, São Paulo, Brazil

    Tomás Zecchini Barrese

  3. University of Campinas, Universidade de Campinas, Campinas, São Paulo, Brazil

    Leandro Luiz Lopes de Freitas & Marcia Torresan Delamain

  4. Hospital São Paulo-University, Universidade Federal de São Paulo, São Paulo, Brazil

    Kleber Simões do Espirito Santo & Otávio César Carvalho Guimarães Baiocchi

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  1. Julia Mota Leite
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  4. Leandro Luiz Lopes de Freitas
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Correspondence to Julia Mota Leite.

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Leite, J.M., Barrese, T.Z., Sementilli, L. et al. Clinical and histopathology characteristics of Castleman disease: a multicenter study of 51 Brazilian patients. Ann Hematol 102, 1121–1129 (2023). https://doi.org/10.1007/s00277-023-05116-6

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  • DOI: https://doi.org/10.1007/s00277-023-05116-6

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