Longitudinal, natural history study reveals the disease burden of idiopathic multicentric Castleman disease
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - year:2024 |
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| Enthalten in: |
Haematologica - (2024) vom: 11. Jan. |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Bustamante, Mateo Sarmiento [VerfasserIn] |
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Date Revised 26.03.2024 published: Print-Electronic Citation Status Publisher |
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| doi: |
10.3324/haematol.2023.283603 |
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| PPN (Katalog-ID): |
NLM366961136 |
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| 245 | 1 | 0 | |a Longitudinal, natural history study reveals the disease burden of idiopathic multicentric Castleman disease |
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| 520 | |a 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 | ||
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| 700 | 1 | |a Pierson, Sheila K |e verfasserin |4 aut | |
| 700 | 1 | |a Ren, Yue |e verfasserin |4 aut | |
| 700 | 1 | |a Bagg, Adam |e verfasserin |4 aut | |
| 700 | 1 | |a Brandstadter, Joshua D |e verfasserin |4 aut | |
| 700 | 1 | |a Srkalovic, Gordan |e verfasserin |4 aut | |
| 700 | 1 | |a Mango, Natalie |e verfasserin |4 aut | |
| 700 | 1 | |a Alapat, Daisy |e verfasserin |4 aut | |
| 700 | 1 | |a Lechowicz, Mary Jo |e verfasserin |4 aut | |
| 700 | 1 | |a Li, Hongzhe |e verfasserin |4 aut | |
| 700 | 1 | |a Van Rhee, Frits |e verfasserin |4 aut | |
| 700 | 1 | |a Lim, Megan S |e verfasserin |4 aut | |
| 700 | 1 | |a Fajgenbaum, David C |e verfasserin |4 aut | |
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