A Novel Predictive Model for Idiopathic Multicentric Castleman Disease: The International Castleman Disease Consortium Study.

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: Although patients with human herpesvirus 8-negative/human immunodeficiency virus-negative multicentric Castleman disease (iMCD) are heterogeneous in clinical, histologic, and subtyping features, they benefit from empirical treatment with decision largely based on clinical evaluation from clinical symptom and disease severity. This prospective study initiated from 176 patients with iMCD interestingly showed that five clinical factors, including age > 40 years, plasmacytic variant subtype, hepatomegaly and/or splenomegaly, hemoglobin <80 g/L, and pleural effusion, could stratify patients into three risk categories: low risk, intermediate risk, and high risk. Such stratification significantly correlates with the clinical performance status and extent of organ dysfunction in these patients enrolled in the study. The validation set of an additional 197 patients with iMCD organized from The International Castleman Disease Consortium was established to confirm and further refine the results from the initial discovery data set. The proposed novel model fully uses relevant clinical parameters and is able to achieve valuable predictive outcomes and support for optimal therapy selection. PMI...
Source: The Oncologist - Category: Cancer & Oncology Authors: Tags: Oncologist Source Type: research