A Novel Predictive Model for Idiopathic Multicentric Castleman Disease: The International Castleman Disease Consortium Study.
Castleman disease
Idiopathic multicentric Castleman disease
International prognostic index
Prognosis
Risk stratification
Treatment
Journal
The oncologist
ISSN: 1549-490X
Titre abrégé: Oncologist
Pays: England
ID NLM: 9607837
Informations de publication
Date de publication:
11 2020
11 2020
Historique:
received:
23
12
2019
accepted:
21
07
2020
pubmed:
28
8
2020
medline:
22
6
2021
entrez:
28
8
2020
Statut:
ppublish
Résumé
Patients with multicentric Castleman disease (MCD) who are negative for human immunodeficiency virus and human herpesvirus 8 are considered to have idiopathic MCD (iMCD). The clinical presentation of iMCD varies from mild constitutional symptoms to life-threatening symptoms or death. The treatment strategy varies from "watchful waiting" to high-dose chemotherapy. This diverse clinical presentation calls for a classification stratification system that takes into account the severity of the disease. We analyzed the clinical, laboratory, and pathologic abnormalities and treatment outcomes of 176 patients with iMCD (median follow-up duration 12 years) from the U.S. and China to better understand the characteristics and prognostic factors of this disease. This discovery set of iMCD results was confirmed from the validation set composed of additional 197 patients with iMCD organized from The International Castleman Disease Consortium. Using these data, we proposed and validated the iMCD international prognostic index (iMCD-IPI), which includes parameters related to patient characteristics (age > 40 years), histopathologic features (plasma cell variant), and inflammatory consequences of iMCD (hepatomegaly and/or splenomegaly, hemoglobin <80 g/L, and pleural effusion). These five factors stratified patients according to their performance status and extent of organ dysfunction into three broad categories: low risk, intermediate risk, and high risk. The iMCD-IPI score accurately predicted outcomes in the discovery study cohort, and the results were confirmed on the validation study cohort. This study represents the largest series of studies on patients with iMCD in the field and proposed a novel risk-stratification model for iMCD-IPI that could be used to guide risk-stratified treatment strategies in patients with iMCD. Patients with idiopathic multicentric Castleman disease (iMCD) can benefit from care based on clinical symptoms and disease severity. This study in 176 patients with iMCD constructed an iMCD-IPI score based on five clinical factors, including age >40 years, plasmacytic variant subtype, hepatomegaly and/or splenomegaly, hemoglobin <80 g/L, and pleural effusion, and stratified patients into three risk categories: low risk, intermediate risk, and high risk. The predictive value was validated in an independent set of 197 patients with iMCD from The International Castleman Disease Consortium. The proposed novel model is valuable for predicting clinical outcome and selecting optimal therapies using clinical parameters.
Sections du résumé
BACKGROUND
Patients with multicentric Castleman disease (MCD) who are negative for human immunodeficiency virus and human herpesvirus 8 are considered to have idiopathic MCD (iMCD). The clinical presentation of iMCD varies from mild constitutional symptoms to life-threatening symptoms or death. The treatment strategy varies from "watchful waiting" to high-dose chemotherapy. This diverse clinical presentation calls for a classification stratification system that takes into account the severity of the disease.
SUBJECTS, MATERIALS, AND METHODS
We analyzed the clinical, laboratory, and pathologic abnormalities and treatment outcomes of 176 patients with iMCD (median follow-up duration 12 years) from the U.S. and China to better understand the characteristics and prognostic factors of this disease. This discovery set of iMCD results was confirmed from the validation set composed of additional 197 patients with iMCD organized from The International Castleman Disease Consortium.
RESULTS
Using these data, we proposed and validated the iMCD international prognostic index (iMCD-IPI), which includes parameters related to patient characteristics (age > 40 years), histopathologic features (plasma cell variant), and inflammatory consequences of iMCD (hepatomegaly and/or splenomegaly, hemoglobin <80 g/L, and pleural effusion). These five factors stratified patients according to their performance status and extent of organ dysfunction into three broad categories: low risk, intermediate risk, and high risk. The iMCD-IPI score accurately predicted outcomes in the discovery study cohort, and the results were confirmed on the validation study cohort.
CONCLUSION
This study represents the largest series of studies on patients with iMCD in the field and proposed a novel risk-stratification model for iMCD-IPI that could be used to guide risk-stratified treatment strategies in patients with iMCD.
IMPLICATIONS FOR PRACTICE
Patients with idiopathic multicentric Castleman disease (iMCD) can benefit from care based on clinical symptoms and disease severity. This study in 176 patients with iMCD constructed an iMCD-IPI score based on five clinical factors, including age >40 years, plasmacytic variant subtype, hepatomegaly and/or splenomegaly, hemoglobin <80 g/L, and pleural effusion, and stratified patients into three risk categories: low risk, intermediate risk, and high risk. The predictive value was validated in an independent set of 197 patients with iMCD from The International Castleman Disease Consortium. The proposed novel model is valuable for predicting clinical outcome and selecting optimal therapies using clinical parameters.
Identifiants
pubmed: 32852137
doi: 10.1634/theoncologist.2019-0986
pmc: PMC7648372
doi:
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
963-973Informations de copyright
© AlphaMed Press 2020.
Références
Blood. 2019 Apr 18;133(16):1720-1728
pubmed: 30760451
Blood. 2014 May 8;123(19):2924-33
pubmed: 24622327
Blood. 2017 Mar 23;129(12):1646-1657
pubmed: 28087540
J Pediatr Hematol Oncol. 2008 Dec;30(12):920-4
pubmed: 19131781
Cancer Control. 2014 Oct;21(4):266-78
pubmed: 25310208
Ann Hematol. 2018 Sep;97(9):1641-1647
pubmed: 29732477
Br J Haematol. 2019 Jan;184(2):232-241
pubmed: 30203839
Oncologist. 2011;16(9):1316-24
pubmed: 21765191
Am J Hematol. 2012 Nov;87(11):997-1002
pubmed: 22791417
Curr Res Transl Med. 2018 Sep;66(3):83-86
pubmed: 30108026
Blood. 2013 Dec 19;122(26):4189-98
pubmed: 24174627
Pathol Int. 2001 Sep;51(9):671-9
pubmed: 11696169
Lancet Oncol. 2014 Aug;15(9):966-74
pubmed: 25042199
Ann Surg. 2012 Apr;255(4):677-84
pubmed: 22367441
Blood. 2017 Mar 23;129(12):1658-1668
pubmed: 28100459
Hematol Oncol Clin North Am. 2018 Feb;32(1):1-10
pubmed: 29157611
Int J Hematol. 2009 Oct;90(3):392-396
pubmed: 19756920
N Engl J Med. 1954 Sep 2;251(10):396-400
pubmed: 13194083
Am J Hematol. 2016 Feb;91(2):220-6
pubmed: 26805758
Oncotarget. 2015 Oct 6;6(30):30408-19
pubmed: 26327301
Blood. 2018 Nov 15;132(20):2115-2124
pubmed: 30181172
Immunotherapy. 2016;8(1):17-26
pubmed: 26634298
Blood. 2014 Dec 4;124(24):3544-52
pubmed: 25331113
Br J Haematol. 2018 Jan;180(2):206-216
pubmed: 29143319