The disease course of Castleman disease patients with fatal outcomes in the ACCELERATE registry
© 2022 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd..
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.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2022 |
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| Erschienen: |
2022 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:198 |
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| Enthalten in: |
British journal of haematology - 198(2022), 2 vom: 24. Juli, Seite 307-316 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Fajgenbaum, David C [VerfasserIn] |
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| Links: |
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| Themen: |
ACCELERATE |
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| Anmerkungen: |
Date Completed 18.07.2022 Date Revised 15.10.2022 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1111/bjh.18214 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
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| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2022 The Authors. British Journal of Haematology published by British Society for Haematology and John Wiley & Sons Ltd. | ||
| 520 | |a 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 | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Research Support, Non-U.S. Gov't | |
| 650 | 4 | |a ACCELERATE | |
| 650 | 4 | |a disease course | |
| 650 | 4 | |a idiopathic multicentric Castleman disease | |
| 650 | 4 | |a mortality | |
| 650 | 4 | |a registry | |
| 700 | 1 | |a Pierson, Sheila K |e verfasserin |4 aut | |
| 700 | 1 | |a Kanhai, Karan |e verfasserin |4 aut | |
| 700 | 1 | |a Bagg, Adam |e verfasserin |4 aut | |
| 700 | 1 | |a Alapat, Daisy |e verfasserin |4 aut | |
| 700 | 1 | |a Lim, Megan S |e verfasserin |4 aut | |
| 700 | 1 | |a Lechowicz, Mary Jo |e verfasserin |4 aut | |
| 700 | 1 | |a Srkalovic, Gordan |e verfasserin |4 aut | |
| 700 | 1 | |a Uldrick, Thomas S |e verfasserin |4 aut | |
| 700 | 1 | |a van Rhee, Frits |e verfasserin |4 aut | |
| 700 | 0 | |a ACCELERATE Registry Team |e verfasserin |4 aut | |
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