Castleman disease

Nature Reviews Disease Primers, Journal Year: 2021, Volume and Issue: 7(1)

Published: Nov. 25, 2021

Language: Английский

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Castleman disease and TAFRO syndrome

Annals of Hematology, Journal Year: 2022, Volume and Issue: 101(3), P. 485 - 490

Published: Jan. 19, 2022

Language: Английский

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Longitudinal, natural history study reveals the disease burden of idiopathic multicentric Castleman disease

Haematologica, Journal Year: 2024, Volume and Issue: unknown

Published: Jan. 11, 2024

Idiopathic multicentric Castleman disease (iMCD) is a rare hematologic disorder with heterogeneous presentations ranging from moderate constitutional symptoms to life-threatening multiorgan system involvement. iMCD patients present vastly different clinical subtypes, some demonstrating thrombocytopenia, anasarca, fever/elevated C-reactive protein, reticulin fibrosis/renal failure, and organomegaly (TAFRO) others more mild/moderate potential for severe (not otherwise specified, NOS). Due its rarity heterogeneity, the natural history long-term burden of are poorly understood. We investigated real-world medical data ACCELERATE, large registry patients, better characterize experienced by these patients. found that iMCD-TAFRO face significant hospitalization burden, requiring time in hospital than iMCD-NOS during year surrounding diagnosis (median [IQR] 36 [18, 61] days vs. 0 [0, 4] days; p

Language: Английский

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A Phase Ib Investigator-Initiated Trial of Filgotinib in Patients With Idiopathic Multicentric Castleman Disease

Cureus, Journal Year: 2025, Volume and Issue: unknown

Published: Feb. 11, 2025

Background Idiopathic multicentric Castleman disease (iMCD) is a chronic systemic inflammatory characterized by the production of interleukin (IL)-6. The contribution Janus kinase, downstream IL-6 signaling, to pathophysiology iMCD has been suggested several studies. Patients and methods This phase Ib single-arm trial evaluated safety profile efficacy filgotinib, JAK1 preferential inhibitor, in patients with iMCD. We recruited activity based on their values C-reactive protein (CRP), hemoglobin, albumin, Eastern Cooperative Oncology Group performance status (ECOG-PS). Filgotinib (200 mg daily) was administered for eight weeks. Results Five who were newly diagnosed or under treatment recruited. lymph node histology all five plasma-cell type. demonstrated favorable manageable adverse events. At weeks, improvements ECOG-PS observed two patients, but no CRP, albumin levels observed. Conclusion filgotinib against comparable those rheumatoid arthritis ulcerative colitis over short duration, not evident after Long-term evaluations are necessary.

Language: Английский

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TAFRO syndrome is associated with anti-SSA/Ro60 antibodies, in contrast to idiopathic castleman disease

Scientific Reports, Journal Year: 2024, Volume and Issue: 14(1)

Published: Feb. 5, 2024

Abstract TAFRO syndrome is an acute systemic inflammatory disease characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis/renal dysfunction, and organomegaly. There have been increasing reports that a distinct from idiopathic multicentric Castleman patients may be positive for anti-SSA antibodies. To assess antibody positivity the clinical characteristics of two diseases, we retrospectively compared 7 10 iMCD in our hospital. The mean age onset was 48.0 (interquartile range [IQR], 41–53) 45.0 (IQR, 35–53) years, respectively. groups had 6 (86%) 4 (40%) male patients, respectively, following pretreatment laboratory values: platelet count, 3.8 2.2–6.4) 35.5 22.2–42.8) × /μL, respectively; C-reactive protein, 10.2 6.8–21.4) 9.5 6.2–13.6) mg/dL, IgG, 1431 1112–1815) 4725 3755–5121) RNA immunoprecipitation (5 cases anti-SSA) or protein array anti-SSA/Ro60) detected antibodies six but not patients; it did detect anti-SSB any patients. None were diagnosed with Sjögren syndrome. All treated tocilizumab (TCZ) responded well. Meanwhile, TCZ showed inadequate responses; thus, both switched to rituximab, which they achieved remission. different features. categorized as severe phenotype

Language: Английский

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Idiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis

Lara D. Veeken, Journal Year: 2022, Volume and Issue: 62(4), P. 1426 - 1435

Published: Aug. 23, 2022

Abstract Idiopathic multicentric Castleman disease (iMCD) is an infrequent and life-threatening disorder characterized by systemic inflammatory symptoms, generalized lymphadenopathy, polyclonal lymphocyte proliferation organ dysfunction caused a hyperinflammatory state. It accounts for one-third to one-half of all (MCD) cases. iMCD often associated with autoimmune manifestations that may precede the diagnosis, be identified at same time or follow it. In addition, also coincide number diseases (such as psoriasis myasthenia gravis) autoinflammatory familial Mediterranean fever). Moreover, diverse disorders, such rheumatoid arthritis, lupus erythematosus, adult-onset Still disease, juvenile idiopathic immunoglobulin (IgG4) related recently described VEXAS syndrome, can present clinical features lymphadenopathy histopathological ‘Castleman-like’ findings compatible those iMCD. Given heterogeneity overlap other diagnosis challenging. this review, we explore between provide practical guidance on in order avoid misdiagnosis confusion conditions.

Language: Английский

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Historical and pathological overview of Castleman disease

Journal of Clinical and Experimental Hematopathology, Journal Year: 2022, Volume and Issue: 62(2), P. 60 - 72

Published: Jan. 1, 2022

Castleman disease consists of several lymphoproliferative subtypes that share some histological features in the lymph nodes. On other hand, numerous clinical findings and etiologies make challenging to understand. The origin is hyaline vascular-type unicentric (UCD), first reported by Benjamin et al. 1954. Although UCD characterized localized lesions lack symptoms, multicentric (MCD) with multiple systemic symptoms was Frizzera 1983. MCD further divided according KSHV/HHV8 infection status. In KSHV/HHV8-related MCD, viral signals lead excessive cytokine production, cause pathologic abnormalities. Some cases plasma cell-type KSHV/HHV8-negative can be found association POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-proteins, skin changes), which a paraneoplastic syndrome. others are idiopathic currently considered heterogeneous group diseases overlapping pathological features. this article, we summarize historical evolution help understand concept. We also review latest ideas definitions within spectrum histopathological findings.

Language: Английский

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Idiopathic Plasmacytic Lymphadenopathy Forms an Independent Subtype of Idiopathic Multicentric Castleman Disease

International Journal of Molecular Sciences, Journal Year: 2022, Volume and Issue: 23(18), P. 10301 - 10301

Published: Sept. 7, 2022

Idiopathic multicentric Castleman disease (iMCD) is a type of that not related to KSHV/HHV8 infection. Currently, iMCD classified into iMCD-TAFRO (thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly) iMCD-NOS (not otherwise specified). The former has been established as relatively homogeneous unit recently re-defined, while the latter considered be heterogeneous could further divided several subtypes. In 1980, Mori et al. proposed concept idiopathic plasmacytic lymphadenopathy (IPL), presenting with polyclonal hypergammaglobulinemia sheet-like proliferation mature plasma cells in lymph nodes. Some researchers consider IPL part iMCD-NOS, although it clearly defined date. This first paper analyze clinicopathologically, examine whether forms uniform iMCD. Histologically, group showed prominent plasmacytosis hyperplasia germinal centers, non-IPL vascularity. Clinically, significant thrombocytosis elevated serum IgG levels compared (p = 0.007, p < 0.001, respectively). Pleural effusion ascites were less common 0.001). was more likely have an indolent clinical course good response anti-IL-6 receptor antibody, counterpart frequently required aggressive medical interventions. Thus, clinicopathologically entity independent subtype

Language: Английский

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CXCL13 is a predictive biomarker in idiopathic multicentric Castleman disease

Nature Communications, Journal Year: 2022, Volume and Issue: 13(1)

Published: Nov. 24, 2022

Idiopathic multicentric Castleman disease (iMCD) is a rare and poorly-understood cytokine storm-driven inflammatory disorder. Interleukin-6 (IL-6) known driver in some patients, but anti-IL-6 therapy with siltuximab not effective all biomarkers indicating success at an early time point following treatment initiation are lacking. Here we show, by comparison of levels 1,178 proteins sera healthy participants (N = 42), patients iMCD 88), related diseases 60), comprehensive landscape candidate mediators predictors response. C-X-C Motif Chemokine Ligand-13 (CXCL13) identified validated as the protein most prominently up-regulated iMCD. Early significant decrease CXCL13 clearly distinguishes responders from non-responders; 17% reduction day 8 predictive response later points. Our study thus suggests that biomarker to

Language: Английский

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Immune‐mediated thrombocytopenia and IL‐6‐mediated thrombocytosis observed in idiopathic multicentric Castleman disease

British Journal of Haematology, Journal Year: 2024, Volume and Issue: 204(3), P. 921 - 930

Published: Jan. 2, 2024

Summary Idiopathic multicentric Castleman disease (iMCD) is a rare haematological disorder characterized by generalized lymphadenopathy with atypical histopathological features and systemic inflammation caused cytokine storm involving interleukin‐6 (IL‐6). Three clinical subtypes are recognized: thrombocytopenia, anasarca, fever, renal dysfunction, organomegaly (iMCD‐TAFRO); idiopathic plasmacytic (iMCD‐IPL), thrombocytosis hypergammaglobulinaemia; iMCD‐not otherwise specified (iMCD‐NOS), which includes patients who do not meet criteria for the other subtypes. Disease pathogenesis poorly understood, potential involvement of infectious, clonal and/or autoimmune mechanisms. To better characterize iMCD clinicopathology gain mechanistic insights into iMCD, we analysed complete blood counts, laboratory values smear morphology among 63 grouped subtype. Patients iMCD‐TAFRO had large platelets, severity associated lower platelet counts transfusion‐resistant similar to what observed immune‐mediated destruction platelets in immune thrombocytopenic purpura. Conversely, elevated iMCD‐IPL were IL‐6 declined following anti‐IL‐6 therapy. Our data suggest that mechanisms contribute thrombocytopenia at least portion whereas drives iMCD‐IPL, these likely pathogenesis.

Language: Английский

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