ArticleJournal of clinical and experimental hematopathology : JCEH2022
Idiopathic multicentric Castleman disease with positive antiphospholipid antibody: atypical and undiagnosed autoimmune disease?
Article in Journal of clinical and experimental hematopathology : JCEH, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 8 papers.
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Who cites it
8 citing papers in PubMed, 10 citations in OpenAlex.
- Elucidating the grading intricacies of idiopathic multicentric Castleman disease histopathology: a pathologist's perspective.American journal of clinical pathology · 2026Review
- Plasma Cell-rich Nephritis with a Systemic Polyclonal Lymphoproliferative Disorder, Mimicking Idiopathic Multicentric Castleman Disease, Systemic Lupus Erythematosus, and IgG4-related Disease.Internal medicine (Tokyo, Japan) · 2025Article
- The Involvement of PI3K-Akt Signaling in the Clinical and Pathological Findings of Idiopathic Multicentric Castleman Disease-Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis, and Organomegaly and Not Otherwise Specified Subtypes.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2025Article
- Transcriptome analysis of the cytokine storm-related genes among the subtypes of idiopathic multicentric Castleman disease.Journal of clinical and experimental hematopathology : JCEH · 2024Article
- TAFRO Syndrome: A Syndrome or a Subtype of Multicentric Castleman Disease?Biomedicines · 2024Article
- Diagnostic challenges in patients with Castleman disease, a single center experience from Hungary.Pathology oncology research : POR · 2024Article
- Idiopathic Plasmacytic Lymphadenopathy Forms an Independent Subtype of Idiopathic Multicentric Castleman Disease.International journal of molecular sciences · 2022Article
- Historical and pathological overview of Castleman disease.Journal of clinical and experimental hematopathology : JCEH · 2022Article
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Authors and funding
12 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Idiopathic multicentric Castleman disease (iMCD) is a systemic disorder characterized by systemic inflammation and organ dysfunction associated with an increase in pro-inflammatory cytokines. Some patients with iMCD are positive for autoantibodies, although their significance and relationship with specific associated autoimmune diseases are unclear. This study retrospectively analyzed the clinicopathological features of iMCD patients focusing on autoantibodies. Among 63 iMCD patients in our database, 19 were positive for at least one autoantibody. Among the 19, we identified five with plasma cell type (PC)-iMCD lymph node histopathology and positive anti-phospholipid antibodies. These patients were likely to have thrombocytopenia, anasarca, fever, reticulin fibrosis or renal insufficiency, organomegaly (TAFRO) symptoms, and thrombotic events. The present study suggests that patients with undiagnosed or atypical autoimmune diseases, including anti-phospholipid syndrome (APS), were treated for iMCD. APS may present with thrombocytopenia or even multi-organ failure, which overlap with clinical presentations of iMCD. Due to differences in the treatment regimen and follow-up, recognition of the undiagnosed autoimmune disease process in those suspected of iMCD is essential. Our study highlights the importance of complete exclusion of differential diagnoses in patients with iMCD in their diagnostic workup.
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