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The disease course of Castleman disease patients with fatal outcomes in the ACCELERATE registry
Castleman disease (CD) describes a group of rare, potentially fatal lymphoproliferative disorders. To determine factors associated with mortality in CD, we analysed data from deceased patients in the ACCELERATE registry and compared them with matched controls. We analysed demographic, treatment and...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
John Wiley and Sons Inc.
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9544190/ https://www.ncbi.nlm.nih.gov/pubmed/35507638 http://dx.doi.org/10.1111/bjh.18214 |
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author | Fajgenbaum, David C. Pierson, Sheila K. Kanhai, Karan Bagg, Adam Alapat, Daisy Lim, Megan S. Lechowicz, Mary Jo Srkalovic, Gordan Uldrick, Thomas S. van Rhee, Frits |
author_facet | Fajgenbaum, David C. Pierson, Sheila K. Kanhai, Karan Bagg, Adam Alapat, Daisy Lim, Megan S. Lechowicz, Mary Jo Srkalovic, Gordan Uldrick, Thomas S. van Rhee, Frits |
author_sort | Fajgenbaum, David C. |
collection | PubMed |
description | Castleman disease (CD) describes a group of rare, potentially fatal lymphoproliferative disorders. To determine factors associated with mortality in CD, we analysed data from deceased patients in the ACCELERATE registry and compared them with matched controls. We analysed demographic, treatment and laboratory data from all deceased CD patients, matched controls and a subgroup of idiopathic multicentric Castleman disease (iMCD) patients. Of the 140 patients in ACCELERATE with a confirmed CD diagnosis, 10 had died. There were 72 patients with confirmed iMCD; six were deceased. The deceased CD cohort had more hospitalisations per year, higher overall hospitalisations and more days hospitalised per month, and received more treatment regimens per year than the matched‐control group. Analysis of laboratory values showed a significantly decreased absolute lymphocyte count at months 3 and 6 in the deceased cohort compared with controls. Among iMCD patients, there was a higher proportion of iMCD‐TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction and organomegaly) cases in the deceased group. The deceased iMCD group had significantly lower immunoglobulin M, international normalised ratio and platelet count. These data demonstrate that there may be differences between patients who have fatal and non‐fatal outcomes, and provide preliminary suggestions for parameters to evaluate further. |
format | Online Article Text |
id | pubmed-9544190 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | John Wiley and Sons Inc. |
record_format | MEDLINE/PubMed |
spelling | pubmed-95441902022-10-08 The disease course of Castleman disease patients with fatal outcomes in the ACCELERATE registry Fajgenbaum, David C. Pierson, Sheila K. Kanhai, Karan Bagg, Adam Alapat, Daisy Lim, Megan S. Lechowicz, Mary Jo Srkalovic, Gordan Uldrick, Thomas S. van Rhee, Frits Br J Haematol Haematological Malignancy‐clinical Castleman disease (CD) describes a group of rare, potentially fatal lymphoproliferative disorders. To determine factors associated with mortality in CD, we analysed data from deceased patients in the ACCELERATE registry and compared them with matched controls. We analysed demographic, treatment and laboratory data from all deceased CD patients, matched controls and a subgroup of idiopathic multicentric Castleman disease (iMCD) patients. Of the 140 patients in ACCELERATE with a confirmed CD diagnosis, 10 had died. There were 72 patients with confirmed iMCD; six were deceased. The deceased CD cohort had more hospitalisations per year, higher overall hospitalisations and more days hospitalised per month, and received more treatment regimens per year than the matched‐control group. Analysis of laboratory values showed a significantly decreased absolute lymphocyte count at months 3 and 6 in the deceased cohort compared with controls. Among iMCD patients, there was a higher proportion of iMCD‐TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis, renal dysfunction and organomegaly) cases in the deceased group. The deceased iMCD group had significantly lower immunoglobulin M, international normalised ratio and platelet count. These data demonstrate that there may be differences between patients who have fatal and non‐fatal outcomes, and provide preliminary suggestions for parameters to evaluate further. John Wiley and Sons Inc. 2022-05-04 2022-07 /pmc/articles/PMC9544190/ /pubmed/35507638 http://dx.doi.org/10.1111/bjh.18214 Text en © 2022 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd. https://creativecommons.org/licenses/by-nc/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes. |
spellingShingle | Haematological Malignancy‐clinical Fajgenbaum, David C. Pierson, Sheila K. Kanhai, Karan Bagg, Adam Alapat, Daisy Lim, Megan S. Lechowicz, Mary Jo Srkalovic, Gordan Uldrick, Thomas S. van Rhee, Frits The disease course of Castleman disease patients with fatal outcomes in the ACCELERATE registry |
title | The disease course of Castleman disease patients with fatal outcomes in the ACCELERATE registry |
title_full | The disease course of Castleman disease patients with fatal outcomes in the ACCELERATE registry |
title_fullStr | The disease course of Castleman disease patients with fatal outcomes in the ACCELERATE registry |
title_full_unstemmed | The disease course of Castleman disease patients with fatal outcomes in the ACCELERATE registry |
title_short | The disease course of Castleman disease patients with fatal outcomes in the ACCELERATE registry |
title_sort | disease course of castleman disease patients with fatal outcomes in the accelerate registry |
topic | Haematological Malignancy‐clinical |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9544190/ https://www.ncbi.nlm.nih.gov/pubmed/35507638 http://dx.doi.org/10.1111/bjh.18214 |
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