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Multicentric Castleman disease: Where are we now?
Hao-Wei Wang1, Stefania Pittaluga1, Elaine S Jaffe1
1Hematopathology Section, Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD.
Insights
Multicentric Castleman disease (MCD) is a complex disorder involving abnormal cytokine activity leading to inflammation and enlarged lymph nodes. Understanding its subtypes, including KSHV/HHV8-associated and idiopathic forms, is crucial for diagnosis and treatment.
Area of Science:
- Hematology
- Immunology
- Oncology
Background:
- Multicentric Castleman disease (MCD) presents with diverse clinicopathological features.
- A central mechanism is dysregulated cytokine activity, causing systemic inflammation and lymphadenopathy.
- Histological findings in MCD overlap with unicentric Castleman disease variants.
Purpose of the Study:
- To review evolving concepts and definitions of MCD.
- To summarize current knowledge on MCD histopathology and pathogenesis.
- To differentiate KSHV/HHV8-associated MCD from idiopathic MCD.
Main Methods:
- Literature review of Castleman disease concepts.
- Analysis of pathogenetic mechanisms in MCD.
- Comparison of histological findings across MCD subtypes.
Main Results:
- MCD pathogenesis involves hypercytokinemia, often driven by Kaposi sarcoma-associated herpesvirus/human herpesvirus-8 (KSHV/HHV8) or other mechanisms in idiopathic MCD.
- KSHV/HHV8-associated MCD can lead to lymphoproliferations and lymphoma risk.
- Idiopathic MCD presents with varied clinical and pathological features due to diverse hypercytokinemia causes.
Conclusions:
- MCD is a spectrum of diseases unified by cytokine dysregulation.
- Distinguishing KSHV/HHV8-positive from idiopathic MCD is important for understanding prognosis and treatment.
- Further research into the diverse pathogenetic pathways of idiopathic MCD is warranted.
Abstract:
Multicentric Castleman disease (MCD) encompasses a spectrum of conditions that give rise to overlapping clinicopathological manifestations. The fundamental pathogenetic mechanism involves dysregulated cytokine activity that causes systemic inflammatory symptoms as well as lymphadenopathy. The histological changes in lymph nodes resemble in part the findings originally described in the unicentric forms Castleman disease, both hyaline vascular and plasma cell variants. In MCD caused by Kaposi sarcoma-associated herpesvirus/human herpesvirus-8 (KSHV/HHV8), the cytokine over activity is caused by viral products, which can also lead to atypical lymphoproliferations and potential progression to lymphoma. In cases negative for KSHV/HHV8, so-called idiopathic MCD, the hypercytokinemia can result from various mechanisms, which ultimately lead to different constellations of clinical presentations and varied pathology in lymphoid tissues. In this article, we review the evolving concepts and definitions of the various conditions under the eponym of Castleman disease, and summarize current knowledge regarding the histopathology and pathogenesis of lesions within the MCD spectrum.
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