Castleman disease (CD) represents a heterogeneous group of lymphoproliferative disorders that share well-defined histopathological features. An observational study of patients with CD was conducted. A total of 53 patients had CD: 20 had the unicentric form (UCD) and 33 the multicentric (MCD) variant; 10 of the latter cases were infected with human herpesvirus-8 (HHV-8) and 23 were idiopathic (iMCD). Median age differed between UCD and iMCD (30 49 years, = .004). Males were completely predominant in HHV-8-associated MCD (100%), and females were more frequent in UCD (75 48%, = .06). Relapses were more frequent in iMCD (57 10% UCD, = .002), and mortality was significantly higher in iMCD and the HHV-8-associated form with respect to UCD. We conclude that UCD is a benign disorder of younger ages and female predominance, while iMCD represents a different entity with more disease relapses and higher mortality.
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| http://dx.doi.org/10.1080/10428194.2019.1639168 | DOI Listing |
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Article Synopsis
- A 52-year-old woman was diagnosed with both neuromyelitis optica spectrum disorder (NMOSD) and idiopathic multicentric Castleman disease (iMCD), making this the first reported case of their coexistence.
- Tocilizumab, an antibody that targets the IL-6 receptor, was used to treat the patient and proved effective for both conditions.
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