O alvo da rapamicina em mamíferos, também chamado de alvo mecanístico da rapamicina e, às vezes, de proteína 12 de ligação FK506, é uma quinase que, em humanos, é codificada pelo gene MTOR. O mTOR é um membro da família de proteínas quinases relacionadas à fosfatidilinositol 3-quinase.
Introdução
O que você precisa saber de cara
Doença Castleman multicêntrica idiopática é uma desordem rara com linfadenopatia generalizada, hepatoesplenomegalia, febre e anasarca. Apresenta-se com elevação de fosfatase alcalina e IgG policlonal, além de autoimunidade.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 12 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 27 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
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Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
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6 ensaios clínicos encontrados, 2 ativos.
Publicações mais relevantes
Recovery trajectories of TAFRO syndrome: a single-center case series highlighting prolonged thrombocytopenia and anasarca.
TAFRO syndrome is a rare systemic inflammatory disorder characterized by thrombocytopenia, anasarca, fever or inflammation, reticulin fibrosis or renal dysfunction, and organomegaly. Despite increasing recognition, its natural history, particularly the timeline of organ-specific recovery, remains poorly understood. We retrospectively analyzed seven patients, encompassing nine treatment courses, diagnosed with TAFRO syndrome at our institution. Time to recovery was assessed across four clinical domains-renal dysfunction, systemic inflammation, anasarca, and thrombocytopenia-using predefined objective criteria. The median time to recovery was 7.0 days for renal dysfunction, 21.0 days for systemic inflammation, 24.0 days for anasarca, and 46.0 days for thrombocytopenia. Recovery of thrombocytopenia and anasarca tended to be delayed compared with that of renal dysfunction and systemic inflammation, with thrombocytopenia showing the longest median time to recovery. These findings indicate substantial heterogeneity in organ-specific recovery trajectories in TAFRO syndrome, with hematologic and fluid-related manifestations resolving more slowly than inflammatory or renal abnormalities. Recognition of domain-specific recovery timelines may aid interpretation of treatment responses and support more balanced, individualized management strategies.
Case Report: Endothelial-targeted bridging therapy for a TTP-like phenotype in fulminant iMCD-TAFRO.
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. 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. 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. 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.
Siltuximab as a first-line therapy for idiopathic multicentric Castleman disease: a retrospective analysis based on the SiMuLa study of the Italian regional network.
Idiopathic Multicentric Castleman Disease (iMCD) is a rare, heterogeneous lymphoproliferative disorder characterized by polyclonal lymphoid hyperplasia, systemic inflammatory symptoms, and generalized lymphadenopathy that can result in multiorgan dysfunction. The interleukin-6 (IL-6)-mediated hyperinflammatory state represents the key pathogenic mechanism of iMCD. Currently, first-line therapy is based on Siltuximab, with or without corticosteroids. Siltuximab (SYLVANT) is a chimeric monoclonal antibody that binds soluble human IL-6, forming stable, high-affinity complexes that neutralize its biological activity. Approximately 40-50% of patients fail to achieve a complete response during first-line Siltuximab therapy. This study aimed to retrospectively evaluate real-world outcomes and safety in patients with iMCD treated with first-line Siltuximab outside clinical trials across the Lazio region of Italy. Real-world data were retrospectively collected from patients with iMCD who received at least one dose of Siltuximab as first-line therapy (11 mg/kg every 3 weeks, until disease progression) between 2018 and March 2025 at Hematology Units across the Lazio region, Italy. Treatment responses were assessed according to Castleman Disease Collaborative Network (CDCN) criteria, based on biochemical, radiologic, and clinical parameters. Safety was evaluated using NCI-CTCAE version 5.0. Fourteen patients were included. The median age was 54 years (range, 18-81), and the median treatment duration was 28 months (range, 3-55). The complete remission rate (CRR) was 29%, while the overall response rate (ORR) reached 86%. At data cutoff, 12 of 14 patients (85.7%) were alive. No infusion-related reactions occurred, and the overall safety profile was favorable. This preliminary real-world experience confirms that first-line Siltuximab is effective and well tolerated in patients with iMCD, achieving durable disease control in the majority of cases. These findings support the continued use of Siltuximab as the standard of care and underscore the importance of expanding real-world registries to better define the epidemiology and treatment outcomes of iMCD in Italy.
Elucidating the grading intricacies of idiopathic multicentric Castleman disease histopathology: a pathologist's perspective.
Idiopathic multicentric Castleman disease (iMCD) is a rare condition with a wide range of signs and symptoms that often overlap with other conditions that cause lymphadenopathy. The diagnosis of iMCD remains challenging, even among experts. Our aim is to highlight the heterogeneity of histopathologic features and presentations of iMCD and to provide tools to help diagnose and raise awareness of this rare condition. We review the most relevant clinicopathologic aspects of iMCD and present our process to accurately diagnose this condition using a case-based approach. We describe how to incorporate the iMCD diagnostic criteria, including grading of histomorphology, laboratory, and clinical findings. Here, we describe our system to grade histopathologic features in iMCD. Together with radiologic findings and laboratory and clinical information, this system helps clinicians accurately identify iMCD and its subtypes, leading to more appropriate and timely diagnosis and treatment. The diagnosis of iMCD can be challenging and requires a multidisciplinary team of hematologists, pathologists/hematopathologists, and other specialists. There is a pressing and unmet clinical need for a harmonized system to grade the histopathologic features of iMCD. This condition should be included in the differential diagnosis when other causes of a lymphoproliferative disorder (eg, infections, autoimmune/inflammatory disorders, malignancy) have been ruled out.
Long-term safety and efficacy of filgotinib in patients with idiopathic multicentric Castleman disease: 52-week results from a Phase Ib clinical trial.
To evaluate the long-term safety and exploratory efficacy of filgotinib, a JAK1-preferential inhibitor, in patients with idiopathic multicentric Castleman disease (iMCD) over 52 weeks in a small, single-arm extension study. This Phase Ib single-arm extension study enrolled five patients with iMCD who completed an initial 8 weeks' treatment with filgotinib. Patients received filgotinib 200 mg once daily for an additional 44 weeks (total 52 weeks). The primary endpoints assessed the comprehensive safety parameters. The secondary endpoints were efficacy. All five patients completed the full 52-week treatment period without permanent discontinuation. No serious adverse events or deaths were observed. All adverse events were mild-to-moderate (Grade 1-2), with upper respiratory infections in 4 patients and headache in 3 of 5 patients. Herpes zoster occurred in one patient. At week 52, three of five patients had ≥1-point CHAP score reduction (one additional patient compared with week 8). Median changes from baseline included: CRP -1.85 mg/dL, hemoglobin +0.50 g/dL, albumin +0.30 g/dL, and ECOG-PS 0 points. Filgotinib was well-tolerated over 52 weeks with no serious adverse events reported. Numerical changes in disease activity markers were observed over time, providing preliminary support for the therapeutic concept of JAK inhibition in iMCD.
Publicações recentes
Morphological characteristics of the renal lesion in TAFRO syndrome and POEMS syndrome: a retrospective and multicenter study.
Deciphering the full spectrum of Castleman diseases based on a cohort of 700 patients in a western country.
[The 516th case: hyperinflammatory state, acute renal dysfunction, hepatosplenomegaly with lymphadenopathy, and recurrent cerebral infarctions].
Primary adrenal insufficiency and autoimmune thyroiditis in an adolescent with Castleman-TAFRO syndrome.
Effects of Janus kinase inhibition and interleukin 6 inhibition on serum cytokine/chemokine in idiopathic multicentric Castleman disease.
📚 EuropePMC215 artigos no totalmostrando 193
Recovery trajectories of TAFRO syndrome: a single-center case series highlighting prolonged thrombocytopenia and anasarca.
Immunological medicineInterfollicular Plasmacytosis and Hyperplastic Germinal Centers in Idiopathic Multicentric Castleman Disease, Idiopathic Plasmacytic Lymphadenopathy Subtype.
American journal of hematologyCase Report: Endothelial-targeted bridging therapy for a TTP-like phenotype in fulminant iMCD-TAFRO.
Frontiers in immunologySiltuximab as a first-line therapy for idiopathic multicentric Castleman disease: a retrospective analysis based on the SiMuLa study of the Italian regional network.
Frontiers in oncologyElucidating the grading intricacies of idiopathic multicentric Castleman disease histopathology: a pathologist's perspective.
American journal of clinical pathologyIs "severe" idiopathic multicentric Castleman disease (iMCD)-idiopathic plasmacytic lymphadenopathy (IPL) really severe?
HemaSphereLong-term safety and efficacy of filgotinib in patients with idiopathic multicentric Castleman disease: 52-week results from a Phase Ib clinical trial.
Modern rheumatologySystemic bone destruction as a presentation of idiopathic multicentric Castleman disease.
British journal of haematologyConcurrent Idiopathic Multicentric Castleman Disease with TAFRO and Sjögren's Syndrome in a Young Patient: A Challenging Diagnostic Overlap.
European journal of case reports in internal medicineA Rapid Transition From Thrombocytosis to Thrombocytopenia in Idiopathic Multicentric Castleman Disease.
The Journal of dermatologyThe clinical significance of the Mediterranean fever gene MEFV variants in Castleman disease.
Communications medicineTAFRO syndrome requiring combined IL 6 and IL 1 inhibition: a case report.
Frontiers in immunologyPolyclonal hypergammaglobulinemia, infiltration of the salivary glands, lymphadenopathy, and kidney damage - Mikulicz's disease, Sjögren's syndrome, or Castleman's disease? Case report and overview of differential diagnosis and treatment.
Klinicka onkologie : casopis Ceske a Slovenske onkologicke spolecnostiRuxolitinib in relapsed/refractory TAFRO syndrome: a report of two cases, including one positive for Epstein-Barr virus, and a literature review.
International journal of hematologyIdiopathic Multicentric Castleman Disease-TAFRO: A Potentially Curable Disease?
American journal of hematologyA Placebo-Controlled Exploratory Trial of Sirolimus for Tocilizumab-Resistant Idiopathic Multicentric Castleman Disease: Early Termination and Long-Term Extension Results Based on Descriptive Results From Two Patients.
CureusDysregulated lymphocyte localization in idiopathic multicentric Castleman disease.
bioRxiv : the preprint server for biologyIdiopathic Multicentric Castleman Disease With Severe Eosinophilia and Diffuse Centrilobular Nodule-A Rare Case Report.
Case reports in hematologyPotential link between COVID-19 infection/vaccination and the onsets of TAFRO syndrome and idiopathic multicentric castleman disease.
Immunology lettersSiltuximab monotherapy improves progression free survival compared to rituximab-based therapies in patients with idiopathic multicentric Castleman disease; indirect comparison of studies using single-arm metanalysis method and the generalized linear mixed model.
Annals of hematologyUse of tocilizumab to treat IgA nephropathy complicated by idiopathic multicentric Castleman disease: a case report.
BMC nephrologyIgG4 plasma cells in hematopathology: beyond IgG4-related disease.
Journal of hematopathologyHeterogeneous Presentations of iMCD: A Single-Institution Case Series.
Case reports in hematologyA case of systemic lupus erythematosus complicated by TAFRO syndrome-like conditions: analysis of C-X-C motif chemokine ligand 13 and B-cell activating factor dynamics and the efficacy of combination therapy with cyclosporine and belimumab.
Modern rheumatology case reportsDiagnostic and therapeutic challenges in hyaline-vascular idiopathic multicentric Castleman disease: A case report.
The Journal of international medical researchAnterior mediastinal lesions in TAFRO syndrome: potential clinical significance.
Rheumatology (Oxford, England)Morphological and quantitative CT features of anterior mediastinal lesions in TAFRO syndrome and idiopathic multicentric Castleman disease.
Frontiers in immunologyDistinct interleukin-6 production in IPL and TAFRO subtypes of idiopathic multicentric Castleman disease.
HaematologicaAtypical clinical presentation associated with Castleman disease: a case report and review of the literature.
Frontiers in medicineThe Evolution and Recent Advances in Diagnostic Criteria for Idiopathic Multicentric Castleman Disease.
American journal of hematologyA patient-derived CABIN1 mutation recapitulates features of idiopathic multicentric Castleman disease in a mouse model.
Blood advancesIdiopathic multicentric Castleman disease with TAFRO syndrome: A rare case report.
Indian journal of cancerStriking effectiveness of siltuximab-based treatment in refractory idiopathic multicentric Castleman disease resembling IgG4-related disease.
Journal of chemotherapy (Florence, Italy)EXT1/EXT2-Associated Membranous Nephropathy Secondary to TAFRO Syndrome: A Case Report.
NephronIdiopathic multicentric Castleman disease resembling autoimmune diseases: A case report.
MedicineThe morphological spectrum of Castleman disease and related disorders: a report from the Lymphoma Workshop of the 22nd Meeting of the European Association of Hematopathology.
Virchows Archiv : an international journal of pathologyComplications and Management of Idiopathic Multicentric Castleman Disease.
HCA healthcare journal of medicine[Clinical Characteristic of Castleman Disease with Renal and Orbit Involvement].
Zhongguo shi yan xue ye xue za zhiCurative Gastrectomy for Advanced Gastric Cancer in a Patient with Idiopathic Multicentric Castleman Disease: A Rare Case Report.
Surgical case reportsInternational Consensus Histopathological Criteria for Subtyping Idiopathic Multicentric Castleman Disease Based on Machine Learning Analysis.
American journal of hematology[Sequential treatment with siltuximab and tocilizumab for childhood idiopathic multicentric Castleman disease: a case report].
Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatricsEfficacy of High-Dose Intravenous Anakinra in Pediatric TAFRO Syndrome: Report of Two Cases and Literature Review.
Pediatric blood & cancer[Breast implant-associated anaplastic large cell lymphoma that developed 20 years after breast reconstruction and was successfully treated with chemotherapy].
[Rinsho ketsueki] The Japanese journal of clinical hematologyPrognostic value of serum C-reactive protein in idiopathic multicentric Castleman disease and construction of a prognostic model for patients.
Frontiers in medicineReversible cerebral vasoconstriction syndrome in idiopathic multicentric Castleman disease under treatment with tocilizumab.
BMJ neurology openCastleman Disease-Still More Questions than Answers: A Case Report and Review of the Literature.
Journal of clinical medicineThe 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, IncEfficacy of tocilizumab monotherapy for autoimmune hemolytic anemia associated with idiopathic multicentric Castleman disease: a case-based review.
Rheumatology internationalLRG1, a novel serum biomarker for iMCD disease activity.
Biomarker researchIdiopathic multicentric Castleman disease - TAFRO results in high levels of mTOR activator SVEP1, tissue factor, and endotheliopathy.
Blood vessels, thrombosis & hemostasisCommon connective tissue disorder and anti-cytokine autoantibodies are enriched in idiopathic multicentric castleman disease patients.
Frontiers in immunologyReal-world data of siltuximab for Chinese patients with iMCD: combination with BCD regimen as a potential approach for severe cases.
Annals of hematologyIFN-γ promotes the progression of iMCD by activating inflammatory monocytes.
BloodSpontaneous remission of iMCD-TAFRO: a case report.
Annals of hematologyPlasma 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)Systemic sclerosis presenting TAFRO syndrome-like manifestations including renal glomerular microangiopathy: a case report and literature review.
Immunological medicineIdiopathic multicentric Castleman disease developing after a diagnosis of sarcoidosis: A case report and literature review.
Respiratory medicine case reportsClinical Characteristics of Patients With Idiopathic Multicentric Castleman Disease Complicated by Autoimmune Hemolytic Anemia.
Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae SinicaeDynamics of Serum Cytokines and Chemokines in Patients With Idiopathic Multicentric Castleman Disease: From a Phase Ib Investigator-Initiated Trial of Filgotinib.
CureusA Phase Ib Investigator-Initiated Trial of Filgotinib in Patients With Idiopathic Multicentric Castleman Disease.
CureusTAFRO Syndrome Without Pathology Supporting Castleman Disease: To Be Treated as Idiopathic Multicentric Castleman Disease-TAFRO or a Distinct Disease Entity?
Turkish journal of haematology : official journal of Turkish Society of HaematologySerum Soluble Interleukin-2 Receptor as a Potential Marker for Assessing Disease Activity in the TAFRO Subtype of Idiopathic Multicentric Castleman Disease.
Internal medicine (Tokyo, Japan)A case report of Castleman disease with paraneoplastic pemphigus and bronchiolitis obliterans: Challenges and key takeaways.
International journal of surgery case reportsMassive endobronchial hemorrhage leading to Cardiac arrest during EBUS-TBNA: a case of successful resuscitation.
BMC pulmonary medicineIdiopathic multicentric Castleman disease in children: a single-center retrospective analysis.
BMC pediatricsNo evidence for active viral infection in unicentric and idiopathic multicentric Castleman disease by Viral-Track analysis.
Scientific reportsClinical Characteristics and Prognosis of Pediatric Idiopathic Multicentric Castleman Disease.
American journal of hematologyClinical and Radiologic Differences in Lung Involvement Between IgG4-Related Disease and Plasma Cell-Type Idiopathic Multicentric Castleman Disease.
LungComputed tomography findings of idiopathic multicentric Castleman disease subtypes.
Journal of clinical and experimental hematopathology : JCEHCo-occurrence of Neuromyelitis Optica Spectrum Disorder and Idiopathic Multicentric Castleman Disease Successfully Treated with Tocilizumab.
Internal medicine (Tokyo, Japan)Quality of life after idiopathic multicentric Castleman disease in China: a cross-sectional, multi-center survey of patient reported outcome and caregiver reported outcome.
Orphanet journal of rare diseasesThe Value of a Pet Scan in Selecting the Best Lymph Node to Biopsy, and Confirming the Diagnosis of Idiopathic Multicentric Castleman Disease with HLH And EBV Viremia in a Previously Healthy Adult.
European journal of case reports in internal medicineThe Enigma of Idiopathic Multicentric Castleman Disease: An Elusive Diagnosis.
CureusIdentifying Castleman disease from non-clonal inflammatory causes of generalized lymphadenopathy.
Hematology. American Society of Hematology. Education ProgramIdiopathic Multicentric Castleman Disease Diagnosed after Lower Extremity Venous Thrombosis Mimicking Immunoglobulin G4-related Disease.
Internal medicine (Tokyo, Japan)[Research Advances in Pathogenesis of Idiopathic Multicentric Castleman Disease].
Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae SinicaeRapid Relapse of Idiopathic Multicentric Castleman Disease After Siltuximab Discontinuation in a Case with Complete Remission for More Than 10 Years.
Turkish journal of haematology : official journal of Turkish Society of HaematologyUnicentric Castleman Disease: Updates and Novel Insights Into Spindle Cell Proliferations and Aggressive Forms of a Localized Disease.
International journal of laboratory hematologyTAFRO subtype of idiopathic multicentric Castleman disease in a 22-year-old man.
CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienneSuccessful Treatment of Idiopathic Multicentric Castleman Disease With Rash as the Initial Symptom Using a Rituximab-Based Regimen.
Journal of hematologySiltuximab in Idiopathic Multicentric Castleman Disease: Real-World Experience.
Journal of hematologyTranscriptome analysis of the cytokine storm-related genes among the subtypes of idiopathic multicentric Castleman disease.
Journal of clinical and experimental hematopathology : JCEHPathophysiology, Treatment, and Prognosis of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome: A Review.
Current issues in molecular biologyIdiopathic multicentric Castleman disease.
Journal of cancer research and therapeuticsEfficacy and safety of orelabrutinib in relapsed/refractory idiopathic multicentric Castleman disease: A single-centre, retrospective study.
British journal of haematologyPeculiar hyper vascular manifestations in idiopathic multicentric castleman disease without tafro syndrome: a case report.
Annals of hematologyAsymptomatic multicentric Castleman disease: a potential early stage of idiopathic MCD.
Blood advancesDiagnostic challenges in patients with Castleman disease, a single center experience from Hungary.
Pathology oncology research : PORAn Unusual Case of HHV-8 Negative, Idiopathic, Multicentric Castleman Disease Following Chronic Lymphocytic Leukaemia.
European journal of case reports in internal medicineIdiopathic multicentric Castleman disease with marrow fibrosis and extramedullary hematopoiesis.
European journal of haematologyEpstein-Barr virus-encoded RNA-positive lymphocytes in bone marrow and lymph nodes in an autopsy case of TAFRO syndrome.
Modern rheumatology case reportsIdiopathic multicentric Castleman disease-TAFRO after COVID-19: A case report.
Modern rheumatology case reportsUnraveling the Complexities of Idiopathic Multicentric Castleman Disease and Its Multi-systemic Associations: A Case Report.
CureusscRNA-seq reveals the landscape of immune repertoire of PBMNCs in iMCD.
OncogeneDelayed diagnosis of TAFRO syndrome: A case report.
MedicineIdiopathic Multicentric Castleman Disease With Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Insufficiency, and Organomegaly (TAFRO) Syndrome in a Liver Transplant Recipient.
ACG case reports journalInternal Psychometric Validation of an International Burden of Illness Survey for Idiopathic Multicentric Castleman Disease.
Oncology and therapy[Relapsed/refractory idiopathic multicentric Castleman disease successfully treated with sirolimus: a case report].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhiIncrease in Vascular Endothelial Growth Factor (VEGF) Expression and the Pathogenesis of iMCD-TAFRO.
BiomedicinesTAFRO Syndrome: Guidance for Managing Patients Presenting Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis, Renal Insufficiency, and Organomegaly.
BiomedicinesBiomarkers and Signaling Pathways Implicated in the Pathogenesis of Idiopathic Multicentric Castleman Disease/Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis, Renal Insufficiency, and Organomegaly (TAFRO) Syndrome.
BiomedicinesCorrigendum: Real-practice management and treatment of idiopathic multicentric Castleman disease with siltuximab: a collection of clinical experiences.
Drugs in contextSuccessful treatment of relapsed idiopathic multicentric Castleman disease-idiopathic plasmacytic lymphadenopathy with orelabrutinib monotherapy: A case report.
British journal of haematologyIdiopathic multicentric Castleman disease and connective tissue disorder successfully treated by siltuximab: a pediatric case report.
Translational pediatricsTacrolimus Treatment for TAFRO Syndrome.
BiomedicinesSirolimus is effective for refractory/relapsed idiopathic multicentric Castleman disease: A single-center, retrospective study.
Annals of hematologyRadiological Imaging Findings of Adrenal Abnormalities in TAFRO Syndrome: A Systematic Review.
BiomedicinesCan We Differentiate Between Primary Sjögren Syndrome and Idiopathic Multicentric Castleman Disease Based on the Characteristics of Pulmonary Cysts?
Journal of thoracic imagingVEXAS syndrome: A new mimicker of idiopathic multicentric Castleman disease.
Joint bone spineSuccessful outcome in a case of idiopathic multicentric Castleman disease with atypical lymphadenopathy and kidney injury: Diagnostic challenges and treatment approach-Case report.
SAGE open medical case reportsA prospective study of zanubrutinib, a Bruton tyrosine kinase inhibitor, in relapsed/refractory idiopathic multicentric Castleman disease.
International journal of hematologyTAFRO Syndrome: A Syndrome or a Subtype of Multicentric Castleman Disease?
BiomedicinesAcquired Hemophilia: A Rare Complication of Pediatric Idiopathic Multicentric Castleman Disease.
PediatricsReal-practice management and treatment of idiopathic multicentric Castleman disease with siltuximab: a collection of clinical experiences.
Drugs in contextA Case of Castleman's Disease with a Marked Infiltration of IgG4-Positive Cells in the Renal Interstitium.
Diagnostics (Basel, Switzerland)Diagnostic challenges of the idiopathic plasmacytic lymphadenopathy (IPL) subtype of idiopathic multicentric Castleman disease (iMCD): Factors to differentiate from IgG4-related disease.
Journal of clinical pathologyWhen idiopathic multicentric Castleman disease meets COVID-19: a multicenter retrospective study from China.
Therapeutic advances in hematologyIdiopathic multicentric Castleman disease (iMCD)-idiopathic plasmacytic lymphadenopathy: A distinct subtype of iMCD-not otherwise specified with different clinical features and better survival.
British journal of haematologyRuxolitinib for the Treatment of Refractory Idiopathic Multicentric Castleman Disease: A Case Report.
Turkish journal of haematology : official journal of Turkish Society of HaematologyTAFRO syndrome is associated with anti-SSA/Ro60 antibodies, in contrast to idiopathic castleman disease.
Scientific reportsSuccessful treatment with bortezomib in combination with dexamethasone in a middle-aged male with idiopathic multicentric Castleman's disease: A case report.
Open medicine (Warsaw, Poland)Longitudinal, natural history study reveals the disease burden of idiopathic multicentric Castleman disease.
HaematologicaTherapy of Castleman's disease with siltuximab - case report and review of literature.
Klinicka onkologie : casopis Ceske a Slovenske onkologicke spolecnostiSingle-cell landscape of idiopathic multicentric Castleman disease in identical twins.
BloodImmune-mediated thrombocytopenia and IL-6-mediated thrombocytosis observed in idiopathic multicentric Castleman disease.
British journal of haematologyIdiopathic multicentric Castleman disease: An update in diagnosis and treatment advances.
Blood reviewsLong-Term Tolerance and Efficacy of Siltuximab (Anti-IL-6) in a Young Adult with Idiopathic Multicentric Castleman Disease During COVID-19.
European journal of case reports in internal medicineConcordance between transbronchial lung cryobiopsy and surgical lung biopsy in patients with idiopathic multicentric Castleman disease: A report of four cases.
Respiratory investigationPeripheral helper-T-cell-derived CXCL13 is a crucial pathogenic factor in idiopathic multicentric Castleman disease.
Nature communicationsA challenging diagnosis of idiopathic multicentric Castleman disease with complex systemic presentation: A case report.
Clinical case reportsIdiopathic multicentric castleman's disease mimicking immunoglobulin G4-related disease responding well to Bortezomib: a case report.
BMC nephrologyCase report: A case of acute exacerbation of interstitial pneumonia associated with TAFRO syndrome.
Frontiers in medicineSymptom burden in patients with idiopathic multicentric Castleman disease and its impact on daily life: an international patient and caregiver survey.
EClinicalMedicineTransformation of HHV-8-negative idiopathic multicentric Castleman disease into diffuse large B-cell lymphoma: A case report from Nepal.
Clinical case reportsTreatment consistent with idiopathic multicentric Castleman disease guidelines is associated with improved outcomes.
Blood advancesHodgkin Lymphoma-related Inflammatory Modification-displayed Castleman Disease-like Histological Features and Positron Emission Tomography/Computed Tomography Usefulness for the Differential Diagnosis.
Internal medicine (Tokyo, Japan)Rituximab-bortezomib-dexamethasone induce high response rates in iMCD in clinical practice.
British journal of haematologyExpert consensus on the integrated diagnosis of idiopathic multicentric Castleman disease.
Revista espanola de patologia : publicacion oficial de la Sociedad Espanola de Anatomia Patologica y de la Sociedad Espanola de CitologiaCost-Effectiveness Analysis of Siltuximab for Australian Public Investment in the Rare Condition Idiopathic Multicentric Castleman Disease.
PharmacoEconomics - openUnmet Clinical Needs in the Management of Idiopathic Multicentric Castleman Disease: A Consensus-based Position Paper From an ad hoc Expert Panel.
HemaSphereRadiological and Pathological Features of Cyst Formation in Idiopathic Multicentric Castleman Disease.
Advances in respiratory medicineA case of idiopathic multicentric Castleman disease with pulmonary hyalinizing granuloma-like multiple pulmonary nodules.
Respiratory investigationvan Rhee F, Rosenthal A, Kanhai K, et al. Siltuximab is associated with improved progression-free survival in idiopathic multicentric Castleman disease. Blood Adv. 2022;6(16):4773-4781.
Blood advancesEvolution of Pulmonary Involvement in Idiopathic Multicentric Castleman Disease-Not Otherwise Specified: From Nodules to Cysts or Consolidation.
ChestIdiopathic Multicentric Castleman Disease Presenting With Hypertensive Choroidopathy: A Case Report.
CureusRare forms of idiopathic multicentric Castleman disease presenting with intrahepatic cholestatic jaundice successfully treated with the bortezomib-based regimen: two case reports.
Annals of hematologyIdiopathic multicentric Castleman disease in an adolescent diagnosed following COVID-19 infection.
Pediatric blood & cancerCase report: Importance of early and continuous tocilizumab therapy in nephrotic syndrome associated with idiopathic multicentric Castleman disease: A case series.
Frontiers in medicineFrom Castleman disease histopathological features to idiopathic multicentric Castleman disease: a multiparametric approach to exclude potential iMCD histopathological mimickers.
Journal of clinical pathologyTreatment-resistant idiopathic multicentric Castleman disease with thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly managed with Janus kinase inhibitors: A case report.
MedicineCXCL13 is a predictive biomarker in idiopathic multicentric Castleman disease.
Nature communicationsUtility of renal biopsy in differentiating idiopathic multicentric Castleman disease from IgG4-related disease.
CEN case reportsRefractory idiopathic multicentric Castleman disease responsive to sirolimus therapy.
American journal of hematologySystemic lupus erythematosus mimicking idiopathic multicentric Castleman disease: A diagnostic dilemma.
SAGE open medical case reportsIdiopathic Multicentric Castleman Disease Occurring Shortly after mRNA SARS-CoV-2 Vaccine.
VaccinesOrgan dysfunction, thrombotic events and malignancies in patients with idiopathic multicentric castleman disease: a population-level US health claims analysis.
LeukemiaIdiopathic Multicentric Castleman Disease with Cutaneous Manifestation: Case Report.
Medicina (Kaunas, Lithuania)Idiopathic Plasmacytic Lymphadenopathy Forms an Independent Subtype of Idiopathic Multicentric Castleman Disease.
International journal of molecular sciencesIdiopathic Multicentric Castleman Disease with Strikingly Elevated IgG4 Concentration in the Serum and Abundant IgG4-Positive Cells in the Tissue: A Case Report.
Diagnostics (Basel, Switzerland)How we manage idiopathic multicentric Castleman disease.
Clinical advances in hematology & oncology : H&OAnalysis of albumin as a prognostic factor in HHV-8/HIV-negative Castleman disease from a multicenter study.
Leukemia & lymphomaIdiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis.
Rheumatology (Oxford, England)Optimisation of anti-interleukin-6 therapy: Precision medicine through mathematical modelling.
Frontiers in immunologyDaratumumab for the treatment of refractory idiopathic multicentric Castleman disease: a case report.
Annals of hematologySevere coronavirus disease 2019 in a patient with TAFRO syndrome: A case report.
Clinical infection in practiceLeg-type form of idiopathic multicentric Castleman disease associated with severe lower extremity chronic venous/lymphatic disease.
EJHaemSiltuximab is associated with improved progression-free survival in idiopathic multicentric Castleman disease.
Blood advancesIdiopathic multicentric Castleman disease with arteriolar endotheliopathy and secondary haemophagocytosis.
The Lancet. HaematologyCandidate biomarkers for idiopathic multicentric Castleman disease.
Journal of clinical and experimental hematopathology : JCEHPsoas and Mediastinal Abscesses during Intravenous Tocilizumab Treatment in Multicentric Castleman Disease.
Internal medicine (Tokyo, Japan)A Rare Variant of Idiopathic Multicentric Castleman Disease: TAFRO Syndrome.
Turkish journal of haematology : official journal of Turkish Society of HaematologyLeucine-rich a-2 glycoprotein as a potential biomarker of idiopathic multicentric Castleman disease with pulmonary involvement: a single-center case-control study from Japan.
Journal of thoracic diseaseTAFRO Syndrome: A Disease Requiring Immediate Medical Attention.
Internal medicine (Tokyo, Japan)The disease course of Castleman disease patients with fatal outcomes in the ACCELERATE registry.
British journal of haematologyPost-transplant Complication With TAFRO Features in a Patient With Acute Myeloid Leukemia.
CureusIncreased mTORC2 pathway activation in lymph nodes of iMCD-TAFRO.
Journal of cellular and molecular medicineThe lymph node transcriptome of unicentric and idiopathic multicentric Castleman disease.
HaematologicaInternational definition of iMCD-TAFRO: future perspectives.
Journal of clinical and experimental hematopathology : JCEHHistorical and pathological overview of Castleman disease.
Journal of clinical and experimental hematopathology : JCEH[Multicentric Castlemans disease. Symptoms, diagnostics and therapy].
Vnitrni lekarstviPOEMS Syndrome: Presented as Idiopathic Multicentric Castleman Disease of Plasma Cell Variant for Eight Years and Dramatic Treatment with Siltuximab Followed by Autologous Peripheral Blood Stem Cell Transplantation.
Diagnostics (Basel, Switzerland)Severe Inflammatory Idiopathic Multicentric Castleman's Disease Coexisting with Advanced Renal Cancer: A Case Report.
The Tohoku journal of experimental medicineSiltuximab-Related Favorable Clinical Outcome for a Patient Suffering from Idiopathic Multicentric Castleman Disease.
Case reports in hematologyIdiopathic multicentric Castleman disease treated with siltuximab for 15 years: a case report.
Therapeutic advances in hematologyIdiopathic multicentric Castleman disease with positive antiphospholipid antibody: atypical and undiagnosed autoimmune disease?
Journal of clinical and experimental hematopathology : JCEHCastleman disease and TAFRO syndrome: To improve the diagnostic consciousness is the key.
World journal of clinical casesBone marrow findings of idiopathic Multicentric Castleman disease: A histopathologic analysis and systematic literature review.
Hematological oncologyA prospective, multicenter study of bortezomib, cyclophosphamide, and dexamethasone in relapsed/refractory iMCD.
Leukemia & lymphomaConcurrence of Marjolin's Ulcer in the Lower Limb in a Patient with Idiopathic Multicentric Castleman Disease: A Case Report.
Medicina (Kaunas, Lithuania)Castleman disease and TAFRO syndrome.
Annals of hematologyCutaneous Plasmacytosis and Idiopathic Multicentric Castleman Disease: A Spectrum of Disease?
The American Journal of dermatopathologyIndolent T-Lymphoblastic Proliferation in Idiopathic Multicentric Castleman Disease.
Acta haematologicaMulticentric Castleman disease in a DOCK8-deficient patient with Orf virus infection.
Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and ImmunologyAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Recovery trajectories of TAFRO syndrome: a single-center case series highlighting prolonged thrombocytopenia and anasarca.
- Case Report: Endothelial-targeted bridging therapy for a TTP-like phenotype in fulminant iMCD-TAFRO.
- Siltuximab as a first-line therapy for idiopathic multicentric Castleman disease: a retrospective analysis based on the SiMuLa study of the Italian regional network.
- Elucidating the grading intricacies of idiopathic multicentric Castleman disease histopathology: a pathologist's perspective.
- Long-term safety and efficacy of filgotinib in patients with idiopathic multicentric Castleman disease: 52-week results from a Phase Ib clinical trial.
- Morphological characteristics of the renal lesion in TAFRO syndrome and POEMS syndrome: a retrospective and multicenter study.
- Deciphering the full spectrum of Castleman diseases based on a cohort of 700 patients in a western country.
- [The 516th case: hyperinflammatory state, acute renal dysfunction, hepatosplenomegaly with lymphadenopathy, and recurrent cerebral infarctions].
- Primary adrenal insufficiency and autoimmune thyroiditis in an adolescent with Castleman-TAFRO syndrome.
- Effects of Janus kinase inhibition and interleukin 6 inhibition on serum cytokine/chemokine in idiopathic multicentric Castleman disease.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:570431(Orphanet)
- MONDO:0035838(MONDO)
- GARD:22309(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
