Leukemia & Myeloid Disorders
Non-Hematological Malignancies in Idiopathic Multicentric Castleman Disease Patients: A Matched Cohort Analysis Using a Health Claims-Based Dataset
Idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disorder characterized by a hyperinflammatory syndrome driven primarily by elevated cytokines, particularly interleukin-6 (IL-6). Numerous studies provide evidence that inflammation increases the risk of several cancers. Elevated inflammatory mediators such as cytokines and chemokines can alter the cellular micromilieu and promote tumorigenesis. A systematic literature review of iMCD reported a three-fold increased prevalence of malignancy in iMCD patients (19%) compared to age-matched controls (6%). However, emergence and prevalence of malignancies in iMCD warrants further investigation to inform evidence-based management guidelines. The primary objective of this study was to evaluate the odds of developing non-hematological malignancies and cancer types in iMCD patients.
Proportion of Cases in the iMCD Population vs. the Non-iMCD Population Control
We report significantly higher odds of having non-hematological malignancies in iMCD patients when compared to the non-iMCD cohort. As iMCD is a challenging diagnosis and claims data can include incorrect codes, it is possible that some patients in the iMCD group could have been incorrectly diagnosed or a malignancy incorrectly coded. Further studies are needed to investigate the biological mechanisms of these observed associations, potential management implications of these findings, and whether IL-6 directed therapies can mitigate these outcomes.