title: Is tocilizumab a potential therapeutic option for refractory unicentric Castleman disease?
abstract: Castleman disease is a rare lymphoproliferative disorder with 2 distinctly defined clinical forms. While multicentric Castleman disease (UCD) poses a potential therapeutic challenge, unicentric variant has historically been considered curable with surgical resection. Hence, little is known to guide management of patients with UCD, refractory to surgical resection and combination chemotherapy. We present a case of a patient, negative for HIV and HHV-8, who had an unsuccessful surgical intervention and no response to radiotherapy and chemotherapy. He had severe paraneoplastic pemphigus and was treated with tocilizumab, an anti-interleukin-6 receptor monoclonal antibody that has demonstrated good response rates in multicentric Castleman disease but demonstrated no clinical response despite 2 months of treatment. Our report is the first to describe a lack of response to tocilizumab in the rare setting of refractory UCD and discuss potential for distinct disease biology.
authors.affiliation: Department of Haematology, University Hospitals of Bristol NHS Trust, Bristol, UK.
authors.forename: Muhammad Bilal
authors.identifier: http://orcid.org/0000-0002-1128-0445
authors.initials: MB
authors.lastname: Abid
authors.affiliation: Department of Haematology, University Hospitals of Bristol NHS Trust, Bristol, UK.
authors.forename: Rachel
authors.initials: R
authors.lastname: Peck
authors.affiliation: Division of Otolaryngology and Head and Neck Surgery, John Hopkins School of Medicine, Baltimore, MD, USA.
authors.forename: Muhammad Abbas
authors.initials: MA
authors.lastname: Abid
authors.affiliation: Department of Haematology, University Hospitals of Bristol NHS Trust, Bristol, UK.
authors.forename: Wesam
authors.identifier: http://orcid.org/0000-0002-2901-7979
authors.initials: W
authors.lastname: Al-Sakkaf
authors.affiliation: Department of Cellular Pathology, University Hospitals Bristol NHS Trust, Bristol, UK.
authors.forename: Yuening
authors.initials: Y
authors.lastname: Zhang
authors.affiliation: Department of Dermatology, University Hospitals of Bristol NHS Trust, Bristol, UK.
authors.forename: Giles S
authors.initials: GS
authors.lastname: Dunnill
authors.affiliation: Department of Oral Medicine, University Hospitals of Bristol NHS Trust, Bristol, UK.
authors.forename: Konrad
authors.initials: K
authors.lastname: Staines
authors.affiliation: Department of Radiology, University Hospitals of Bristol NHS Trust, Bristol, UK.
authors.forename: Iara-Maria
authors.initials: IM
authors.lastname: Sequeiros
authors.affiliation: Department of Haematology, University Hospitals of Bristol NHS Trust, Bristol, UK.
authors.forename: Lisa
authors.initials: L
authors.lastname: Lowry
mesh_terms: D061067:Antibodies, Monoclonal, Humanized / Q000008:administration & dosage / Q000494:pharmacology / Q000627:therapeutic use*; D005871:Castleman Disease / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged
chemical_list: D061067:Antibodies, Monoclonal, Humanized; C502936:tocilizumab
keywords: IL-6; cytokine storm; tocilizumab; unicentric Castleman disease (UCD)