Paraneoplastic pemphigus uncovers distinct clinical and biological phenotypes of western unicentric Castleman disease

• Published in: British journal of haematology Vol. 202; no. 2; pp. 267 - 278
• Main Authors: Dieudonné, Yannick, Silvestrini, Marc‐Antoine, Dossier, Antoine, Meignin, Véronique, Jouenne, Fanélie, Mahévas, Thibault, Bouaziz, Jean‐David, Jackson, Margaret A., Mordant, Pierre, Poirot, Justine, Onodi, Fanny, Calvani, Julien, Hourseau, Muriel, Evrard, Diane, Berisha, Mirlinda, Perrin, François, Danel, Claire, Borie, Raphael, Galicier, Lionel, Mourah, Samia, Bengoufa, Djaouida, Oksenhendler, Eric, Grootenboer‐Mignot, Sabine, Boutboul, David
• Format: Journal Article
• Language: English
• Published: England Blackwell Publishing Ltd 01.07.2023
• Subjects: Autoantibodies; Bronchiolitis obliterans; Bronchopneumonia; Castleman's disease; Hematology; Immunoproliferative diseases; Lymphocytes; Medical prognosis; Multivariate analysis; Myasthenia gravis; Neuromuscular junctions; paraneoplastic pemphigus; Pemphigus; periplakin epitope mapping; Phenotypes; Sarcoma; unicentric Castleman disease; periplakin epitope mapping; unicentric Castleman disease; paraneoplastic pemphigus

Summary Unicentric Castleman disease (UCD) is a lymphoproliferative disease of unknown cause. Paraneoplastic pemphigus (PNP) is a major complication shown to be associated with a poor prognosis, with particular severity in patients with bronchiolitis obliterans (BO). This study describes the clinica...