Longitudinal, natural history study reveals the disease burden of idiopathic multicentric Castleman disease
Mateo Sarmiento Bustamante,
Sheila K. Pierson,
Yue Ren,
Adam Bagg,
Joshua D. Brandstadter,
Gordan Srkalovic,
Natalie Mango,
Daisy Alapat,
Mary Jo Lechowicz,
Hongzhe Li,
Frits van Rhee,
Megan S. Lim,
David C. Fajgenbaum
Affiliations
Mateo Sarmiento Bustamante
Center for Cytokine Storm Treatment and Laboratory, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104
Sheila K. Pierson
Center for Cytokine Storm Treatment and Laboratory, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104
Yue Ren
Department of Biostatistics, Epidemiology, and Informatics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104
Adam Bagg
Department of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, PA, 19104
Joshua D. Brandstadter
Division of Hematology/Oncology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104
Gordan Srkalovic
Sparrow Herbert-Herman Cancer Center, Michigan State University College of Human Medicine, Lansing, MI, 48912
Natalie Mango
Center for Cytokine Storm Treatment and Laboratory, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104
Daisy Alapat
Department of Pathology, College of Medicine, University of Arkansas for Medical Sciences, Little Rock, AR, 72205
Mary Jo Lechowicz
Department of Hematology and Medical Oncology, Emory University School of Medicine and Winship Cancer Institute, Atlanta, GA, 30322
Hongzhe Li
Department of Biostatistics, Epidemiology, and Informatics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104
Frits van Rhee
Myeloma Center, Winthrop P. Rockefeller Cancer Institute, University of Arkansas for Medical Sciences, Little Rock, AR, 72205
Megan S. Lim
Department of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, New York City, New York, 10065
David C. Fajgenbaum
Center for Cytokine Storm Treatment and Laboratory, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, 19104
Idiopathic multicentric Castleman disease (iMCD) is a rare hematologic disorder with heterogeneous presentations ranging from moderate constitutional symptoms to life-threatening multiorgan system involvement. iMCD patients present with vastly different clinical subtypes, with some patients demonstrating thrombocytopenia, anasarca, fever/elevated C-reactive protein, reticulin fibrosis/renal failure, and organomegaly (TAFRO) and others demonstrating more mild/moderate symptoms with potential for severe disease (not otherwise specified, NOS). Due to its rarity and heterogeneity, the natural history and long-term burden of iMCD are poorly understood. We investigated real-world medical data from ACCELERATE, a large natural history registry of Castleman disease patients, to better characterize the long-term disease burden experienced by these patients. We found that iMCD-TAFRO patients face significant hospitalization burden, requiring more time in the hospital than iMCD-NOS patients during the year surrounding diagnosis (median [IQR] 36 [18, 61] days vs. 0 [0, 4] days; p
