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Multicentric Castleman disease presenting as a chylous pleural effusion
Danny Epstein1, Yaniv Zohar2, David Ovadya3
1Internal Medicine "B" Department, Rambam Health Care Campus, Haifa, Israel.
Pathology, Research and Practice
|September 20, 2020
Summary
Castleman disease, a rare disorder, can cause chylous pleural effusion. This report details the first hypervascular variant case, successfully treated with anti-interleukin-6 therapy and steroids.
Area of Science:
- Hematology
- Oncology
- Pulmonology
Background:
- Castleman disease is a rare lymphoproliferative disorder often presenting with constitutional symptoms.
- Pleural effusion is a common manifestation, but chylous pleural effusion is exceptionally rare, with only one prior adult case reported.
Observation:
- This report details the first case of a hypervascular variant of Castleman disease presenting with chylous pleural effusion in an adult.
- The patient exhibited symptoms consistent with Castleman disease and significant chylous pleural effusion.
Findings:
- The hypervascular Castleman disease variant was diagnosed based on clinical presentation and imaging characteristics.
- Successful treatment was achieved using a combination of an anti-interleukin-6 agent and corticosteroids.
Implications:
- This case highlights the importance of considering Castleman disease, particularly its hypervascular variant, in patients with chylous pleural effusion.
- The successful treatment underscores the efficacy of targeting interleukin-6 in managing this rare condition, offering a potential therapeutic strategy for similar cases.
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