# Unraveling the Complexities of Idiopathic Multicentric Castleman Disease and Its Multi-systemic Associations: A Case Report

**Authors:** Aadi R Palvia, Prince Saha, Akshay Rahul Nandi, Abhiram Rao Damera, Aditya Suresh

PMC · DOI: 10.7759/cureus.64935 · Cureus · 2024-07-19

## TL;DR

This case report explores a rare disease called idiopathic multicentric Castleman disease in a young woman, highlighting its complex connections with antiphospholipid syndrome and a virus.

## Contribution

The report provides new insights into the potential link between HHV-6 and Castleman disease, and the importance of early APS detection.

## Key findings

- A patient with idiopathic multicentric Castleman disease also had antiphospholipid syndrome and HHV-6 positivity.
- Treatment with siltuximab and tocilizumab was effective, suggesting IL-6's role in the disease.
- The case highlights the plasma cell variant of Castleman disease and its multi-systemic associations.

## Abstract

Castleman disease (CD) comprises a rare spectrum of disorders characterized by benign lymphoepithelial proliferation, classified into unicentric and multicentric forms. The idiopathic multicentric Castleman disease (iMCD) subtype, specifically, is challenging to diagnose and treat due to its variable manifestations and unpredictable disease course. We report a case of a 23-year-old female with a history of iron deficiency anemia presenting with concurrent antiphospholipid syndrome (APS) and human herpesvirus-6 (HHV-6) positivity. Investigations revealed a gastric mass, with a biopsy suggestive of the plasma cell variant of CD. This case report aims to understand the possible association of HHV-6 positivity with CD and the significance of diagnosing APS early in patients with the disease. Treatment with siltuximab and tocilizumab proved effective, highlighting the role of interleukin 6 (IL-6) in the elusive etiology of this condition.

## Linked entities

- **Proteins:** IL6 (interleukin 6)
- **Diseases:** Castleman disease (MONDO:0015564), idiopathic multicentric Castleman disease (MONDO:0035838), antiphospholipid syndrome (MONDO:0017278), iron deficiency anemia (MONDO:0001356)

## Full-text entities

- **Genes:** IL6 (interleukin 6) [NCBI Gene 3569] {aka BSF-2, BSF2, CDF, HGF, HSF, IFN-beta-2}
- **Diseases:** gastric mass (MESH:C536030), APS (MESH:D016736), Idiopathic Multicentric Castleman Disease (MESH:C537372), CD (MESH:D005871), iron deficiency anemia (MESH:D018798)
- **Chemicals:** siltuximab (MESH:C504234), tocilizumab (MESH:C502936)
- **Species:** Homo sapiens (human, species) [taxon 9606], Human betaherpesvirus 6 (species) [taxon 10368]

## Full text

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## Figures

3 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11332968/full.md

## References

13 references — full list in the complete paper: https://tomesphere.com/paper/PMC11332968/full.md

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Source: https://tomesphere.com/paper/PMC11332968