{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,26]],"date-time":"2026-02-26T07:20:02Z","timestamp":1772090402858,"version":"3.50.1"},"reference-count":21,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,2,26]],"date-time":"2026-02-26T00:00:00Z","timestamp":1772064000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>iMCD-TAFRO (the TAFRO clinical subtype of idiopathic multicentric Castleman disease (iMCD)) is characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. Diagnosis is challenging due to its rarity, non-specific early presentation, and overlap with sepsis, lymphoma, and thrombotic microangiopathy (TMA). Endothelial injury is increasingly recognized as a central driver of its severe complications.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>A previously healthy 20-year-old woman presented with rapid progression of fever, anasarca, jaundice, and respiratory failure. Laboratory findings revealed severe thrombocytopenia, microangiopathic hemolytic anemia, markedly elevated IL-6, and critically reduced ADAMTS13 activity (5.41%) without an inhibitor by a Bethesda assay, in a sample obtained before the first therapeutic plasma exchange (TPE) and before any fresh frozen plasma (FFP) infusion, suggesting a cytokine-driven thrombotic thrombocytopenic purpura (TTP)-like syndrome. Extensive workup excluded primary infections and malignancies. Diagnosis of iMCD-TAFRO was confirmed by a lymph-node core biopsy showing features consistent with Castleman disease with plasmacytosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This case highlights that iMCD-TAFRO can manifest as a fulminant, endotheliopathy-dominated syndrome in young adults, mimicking primary TTP. The severe ADAMTS13 deficiency in this context likely results from consumption due to endothelial activation rather than an immune-mediated process. This distinction is critical for appropriate management.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Management and Outcome<\/jats:title>\n                    <jats:p>Prior to definitive diagnosis, an endothelium-directed bundle was initiated, including TPE plus adjunctive FFP infusion, corticosteroids, and anisodamine for microcirculatory support; stabilization occurred in the context of multimodal supportive care. Upon diagnostic confirmation, targeted anti-IL-6 therapy (siltuximab) led to significant clinical improvement. Despite a subsequent relapse with pulmonary hypertension, intensified immunosuppression achieved complete remission at one-year follow-up. This case illustrates a pathophysiology-informed bridging bundle to stabilize endothelial and microcirculatory dysfunction while pursuing definitive diagnosis and targeted cytokine blockade in severe iMCD-TAFRO.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fimmu.2026.1776382","type":"journal-article","created":{"date-parts":[[2026,2,26]],"date-time":"2026-02-26T06:53:12Z","timestamp":1772088792000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Case Report: Endothelial-targeted bridging therapy for a TTP-like phenotype in fulminant iMCD-TAFRO"],"prefix":"10.3389","volume":"17","author":[{"given":"Jing","family":"Du","sequence":"first","affiliation":[{"name":"Department of Emergency Medicine, Sun Yat-sen Memorial Hospital, Sun Yat-sen University","place":["Guangzhou, 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