Raras
Buscar doenças, sintomas, genes...
Síndrome TAFRO
ORPHA:457077CID-10 · M35.8CID-11 · 4A4YDOENÇA RARA

Uma forma da Doença de Castleman multicêntrica, caracterizada por: plaquetas baixas no sangue, acúmulo de líquido na barriga (barriga d'água) e inchaço generalizado, anemia com glóbulos vermelhos pequenos, endurecimento da medula óssea, problemas nos rins e aumento do tamanho de órgãos.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Uma forma da Doença de Castleman multicêntrica, caracterizada por: plaquetas baixas no sangue, acúmulo de líquido na barriga (barriga d'água) e inchaço generalizado, anemia com glóbulos vermelhos pequenos, endurecimento da medula óssea, problemas nos rins e aumento do tamanho de órgãos.

Publicações científicas
298 artigos
Último publicado: 2026 Jan-Dec

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
<1 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Casos conhecidos
28
pacientes catalogados
Início
Adolescent
+ adult, elderly
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: M35.8
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🩸
Sangue
5 sintomas
🫃
Digestivo
3 sintomas
🛡️
Imunológico
2 sintomas
🫘
Rins
2 sintomas
🦴
Ossos e articulações
1 sintomas
🫁
Pulmão
1 sintomas

+ 12 sintomas em outras categorias

Características mais comuns

90%prev.
Concentração elevada de proteína C-reativa circulante
Muito frequente (99-80%)
90%prev.
Hepatoesplenomegalia
Muito frequente (99-80%)
90%prev.
Anasarca
Muito frequente (99-80%)
90%prev.
Trombocitopenia
Muito frequente (99-80%)
90%prev.
Linfadenopatia
Muito frequente (99-80%)
55%prev.
Ascite
Frequente (79-30%)
27sintomas
Muito frequente (5)
Frequente (13)
Ocasional (1)
Muito raro (6)
Sem dados (2)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 27 características clínicas mais associadas, ordenadas por frequência.

Concentração elevada de proteína C-reativa circulanteElevated circulating C-reactive protein concentration
Muito frequente (99-80%)90%
HepatoesplenomegaliaHepatosplenomegaly
Muito frequente (99-80%)90%
Anasarca
Muito frequente (99-80%)90%
TrombocitopeniaThrombocytopenia
Muito frequente (99-80%)90%
LinfadenopatiaLymphadenopathy
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico298PubMed
Últimos 10 anos200publicações
Pico202532 papers
Linha do tempo
2026Hoje · 2026📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

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

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Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Síndrome TAFRO

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Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

Nenhum ensaio clínico registrado para esta condição.

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Publicações mais relevantes

🥉Melhor nível de evidência: Relato de caso
Timeline de publicações
291 papers (10 anos)
#1

Recovery trajectories of TAFRO syndrome: a single-center case series highlighting prolonged thrombocytopenia and anasarca.

Immunological medicine2026 Mar 23

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.

#2

The value of 18F-fluorodeoxyglucose positron emission tomography/computed tomography in the diagnosis and evaluation of TAFRO syndrome: a review of cases and literature.

Clinical radiology2026 Apr

This retrospective case series intends to summarise the 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) imaging characteristics of TAFRO syndrome, providing imaging evidence for its clinical diagnosis and differential diagnosis to enhance early diagnostic accuracy and improve patient prognosis. A retrospective analysis was conducted on the 18F-FDG PET/CT images and clinical data of 5 patients with TAFRO syndrome (3 males and 2 females, with an average age of 51.4 (39-58) years). Combined with domestic and international literature, the value of 18F-FDG PET/CT in the diagnosis and evaluation of TAFRO syndrome was summarised. All the cases were diagnosed as multicentric Castleman disease (MCD) and all showed typical imaging manifestations of TAFRO syndrome. The size of the affected lymph nodes and the maximum standardised uptake value (SUVmax) of the patients were measured. It was found that all showed varying degrees of enlargement accompanied by increased 18F-FDG metabolism (SUVmax > 2.5). In addition, spleen length was measured in the craniocaudal dimension for all patients and it was found that 2 patients had significant enlargement. Based on the analysis of the PET/CT images of the patients, the bone marrow showed varying degrees of metabolic activity, and pleural effusions and ascites were clearly identifiable. 18F-FDG PET/CT imaging, as a functional metabolic imaging technique, can detect metabolic abnormalities at the sub-centimetre level. Moreover, a single examination can cover the entire body, enabling better detection of occult lesions and providing significant assistance in the diagnosis and assessment of TAFRO syndrome.

#3

Concurrent 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 medicine2026

Idiopathic multicentric Castleman disease (iMCD) with TAFRO syndrome (characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, organomegaly) is a recently described disorder. The diagnostic criteria for iMCD-TAFRO exclude patients with concomitant autoimmune diseases such as primary Sjögren's syndrome. However, recent case reports suggest that although rare, these conditions may coexist. We report the case of a 37-year-old woman who presented with escalating systemic inflammation, cytopenias, anasarca, renal dysfunction and organomegaly, with lymph node biopsy suggestive of Castleman disease. Simultaneously, dense lacrimal gland hyperplasia with histopathology demonstrating lymphoepithelial sialadenitis and strongly positive anti-Ro52 antibodies led to a diagnosis of Sjögren's syndrome. Given the rapid clinical deterioration and marked hyperinflammation, corticosteroid therapy and tocilizumab were implemented, leading to sustained remission. This case illustrates the diagnostic complexity posed by overlapping features of Idiopathic multicentric Castleman disease with TAFRO syndrome and Sjögren's syndrome and reinforces the need to reconsider existing diagnostic criteria to ensure timely diagnosis and effective treatment. Epidemiologically, this is also a rare case of iMCD with TAFRO syndrome in a patient of African descent, contrasting with previous reports predominantly involving Asian individuals. Idiopathic multicentric Castleman disease (iMCD) with TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, organomegaly) (iMCD-TAFRO) is a rare, life-threatening disorder marked by severe systemic inflammation and multiorgan failure, and early recognition and timely initiation of immunomodulatory therapy are critical for positive outcomes.Although current diagnostic criteria for iMCD-TAFRO exclude patients with concomitant autoimmune diseases such as Sjögren's syndrome, an increasing number of case reports suggest that these conditions may coexist, challenging existing definitions and suggesting a potential physiopathological overlap.This case highlights the diagnostic challenge posed by the coexistence of these conditions and underscores the need for an individualized assessment integrating clinical, laboratory, and immunological findings.

#4

Correction: TAFRO syndrome requiring combined IL 6 and IL 1 inhibition: a case report.

Frontiers in immunology2026

[This corrects the article DOI: 10.3389/fimmu.2025.1729525.].

#5

Ruxolitinib in relapsed/refractory TAFRO syndrome: a report of two cases, including one positive for Epstein-Barr virus, and a literature review.

International journal of hematology2026 Jan 19

TAFRO syndrome is a rare systemic inflammatory disorder characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly. Its pathogenesis remains elusive, and its non-specific symptoms overlap with idiopathic multicentric Castleman disease, complicating its management. This report discusses two cases of TAFRO syndrome resistant to conventional treatments, including corticosteroids, tocilizumab, and rituximab. Both patients showed significant clinical improvement after treatment with ruxolitinib, a JAK-STAT pathway inhibitor. The first patient was a 55-year-old man who showed clinical improvement with ruxolitinib after the failure of multiple lines of treatment, including corticosteroids, tocilizumab, rituximab, and cyclosporin A. The second was another 55-year-old man who experienced disease relapse 18 months after initial treatment but responded rapidly to ruxolitinib. These cases highlight the potential role of ruxolitinib as a therapeutic option for refractory TAFRO syndrome. In addition, Epstein-Barr virus (EBV) DNA was detected in the serum of the first patient, making this the first report to demonstrate the effectiveness of ruxolitinib in EBV-associated TAFRO syndrome. These findings highlight the need for further studies to confirm the efficacy of ruxolitinib in such cases and elucidate the pathophysiological mechanisms underlying TAFRO syndrome, which could guide future therapeutic strategies.

Publicações recentes

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📚 EuropePMC250 artigos no totalmostrando 194

2026

Recovery trajectories of TAFRO syndrome: a single-center case series highlighting prolonged thrombocytopenia and anasarca.

Immunological medicine
2026

The value of 18F-fluorodeoxyglucose positron emission tomography/computed tomography in the diagnosis and evaluation of TAFRO syndrome: a review of cases and literature.

Clinical radiology
2026

Concurrent 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 medicine
2026

Multicentric Castleman disease (MCD) presenting with retiform purpura: A rare dermatologic manifestation of a systemic disease.

JAAD case reports
2025

TAFRO syndrome requiring combined IL 6 and IL 1 inhibition: a case report.

Frontiers in immunology
2026

Ruxolitinib in relapsed/refractory TAFRO syndrome: a report of two cases, including one positive for Epstein-Barr virus, and a literature review.

International journal of hematology
2026

Potential link between COVID-19 infection/vaccination and the onsets of TAFRO syndrome and idiopathic multicentric castleman disease.

Immunology letters
2026

Polyarteritis nodosa presenting with TAFRO signs following COVID-19 infection: case report.

Modern rheumatology case reports
2025

Heterogeneous Presentations of iMCD: A Single-Institution Case Series.

Case reports in hematology
2025

A 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 reports
2025

Effective Treatment with Ruxolitinib and Ropeginterferon Alfa-2b for Refractory TAFRO-like Syndrome.

Internal medicine (Tokyo, Japan)
2026

Anterior mediastinal lesions in TAFRO syndrome: potential clinical significance.

Rheumatology (Oxford, England)
2026

Tafro syndrome: early combination therapy outcomes and prolonged thrombocytopenia-a seven-case series.

Modern rheumatology
2025

[Recent advances in TAFRO syndrome and Castleman disease].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2025

Morphological and quantitative CT features of anterior mediastinal lesions in TAFRO syndrome and idiopathic multicentric Castleman disease.

Frontiers in immunology
2025

[Weight loss, fever and abdominal pain in a 58 year-old man].

La Revue de medecine interne
2025

TAFRO Syndrome-A Decade Later, Still Racing Against Time.

Biomedicines
2025

Idiopathic multicentric Castleman disease with TAFRO syndrome: A rare case report.

Indian journal of cancer
2025

EXT1/EXT2-Associated Membranous Nephropathy Secondary to TAFRO Syndrome: A Case Report.

Nephron
2025

Thrombocytopenia, anasarca, fever, reticulum fibrosis, and organomegaly syndrome triggered by vaccination against SARS-CoV-2 messenger RNA vaccine.

Modern rheumatology case reports
2025

Multicentric Castleman disease presenting with orbital involvement progressing to TAFRO syndrome: a case report.

BMC ophthalmology
2025

The 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 pathology
2025

[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 pediatrics
2025

Clinical Utility of an Increased Pretreatment Immature Platelet Fraction in the Early Diagnosis of TAFRO Syndrome, Especially in Patients with Late-onset Thrombocytopenia: A Case Series and Literature Review.

Internal medicine (Tokyo, Japan)
2025

Efficacy of High-Dose Intravenous Anakinra in Pediatric TAFRO Syndrome: Report of Two Cases and Literature Review.

Pediatric blood &amp; cancer
2025

Survival and Hospital Discharge Achieved Through Intensive Circulatory, Fluid, and Respiratory Support in Severe TAFRO (Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis, and Organomegaly) Syndrome With Multiorgan Dysfunction: A Case Report.

Cureus
2025

A Series of Glomerular Diseases That Developed After COVID-19 Vaccination.

Cureus
2025

Successful Treatment of Refractory TAFRO Syndrome with Cyclophosphamide: A Report of Two Cases.

Internal medicine (Tokyo, Japan)
2025

Case Report: TAFRO syndrome misdiagnosed as liver cirrhosis due to persistent abdominal distension.

Frontiers in immunology
2025

Successful Treatment of TAFRO (Thrombocytopenia, Anasarca, Fever, Renal Insufficiency, and Organomegaly) Syndrome With Triple Combination Therapy of Corticosteroid, Tocilizumab, and Cyclosporine: A Case Report.

Cureus
2025

First-line treatment of TAFRO syndrome with rituximab: a case report and literature review.

Annals of hematology
2025

Systemic sclerosis presenting TAFRO syndrome-like manifestations including renal glomerular microangiopathy: a case report and literature review.

Immunological medicine
2025

TAFRO Syndrome with Massive Bilateral Adrenal Hemorrhage.

Internal medicine (Tokyo, Japan)
2025

TAFRO 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 Haematology
2025

Myelodysplastic neoplasms with repeating TAFRO syndrome-like symptoms.

International journal of hematology
2025

Exploring the Clinical Diversity of Castleman Disease and TAFRO Syndrome: A Japanese Multicenter Study on Lymph Node Distribution Patterns.

American journal of hematology
2024

Computed tomography findings of idiopathic multicentric Castleman disease subtypes.

Journal of clinical and experimental hematopathology : JCEH
2024

Case report: Atypical POEMS syndrome without polyneuropathy, complicated by borderline TAFRO syndrome.

Frontiers in medicine
2025

Renal involvement in TAFRO syndrome: a review.

Clinical and experimental nephrology
2024

Pathophysiology, Treatment, and Prognosis of Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome: A Review.

Current issues in molecular biology
2024

Peculiar hyper vascular manifestations in idiopathic multicentric castleman disease without tafro syndrome: a case report.

Annals of hematology
2024

Castleman Disease: A Rare Lymphoproliferative Disorder With Diverse Clinical Presentation, Diagnosis, and Treatment Approach.

Cureus
2024

An Unusual Case of HHV-8 Negative, Idiopathic, Multicentric Castleman Disease Following Chronic Lymphocytic Leukaemia.

European journal of case reports in internal medicine
2024

TAFRO syndrome with abdominal pain as the first symptom accompanied by liver damage with hyperbilirubinemia: A case report.

Heliyon
2025

TAFRO-like Symptoms in a Sjögren's Syndrome Patient with HTLV-1 Infection.

Internal medicine (Tokyo, Japan)
2025

Epstein-Barr virus-encoded RNA-positive lymphocytes in bone marrow and lymph nodes in an autopsy case of TAFRO syndrome.

Modern rheumatology case reports
2025

Idiopathic multicentric Castleman disease-TAFRO after COVID-19: A case report.

Modern rheumatology case reports
2024

Delayed diagnosis of TAFRO syndrome: A case report.

Medicine
2024

Pediatric TAFRO syndrome: A multi-institution case series illustrating clinical challenges and excellent outcomes.

Pediatric blood &amp; cancer
2024

Comparison of pathophysiological differences in TAFRO syndrome with or without lymphadenopathy.

Journal of clinical and experimental hematopathology : JCEH
2024

Idiopathic Multicentric Castleman Disease With Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Insufficiency, and Organomegaly (TAFRO) Syndrome in a Liver Transplant Recipient.

ACG case reports journal
2024

Long-term clinicopathological characteristics of TAFRO syndrome and its relapse: a case series study.

Clinical kidney journal
2024

Recurrent Cerebral Hemorrhaging with Platelet Dysfunction Accompanied by Anti-glycoprotein VI Autoantibodies in a Patient with TAFRO Syndrome.

Internal medicine (Tokyo, Japan)
2024

TAFRO Syndrome and COVID-19.

Biomedicines
2024

TAFRO Syndrome: Guidance for Managing Patients Presenting Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis, Renal Insufficiency, and Organomegaly.

Biomedicines
2024

Biomarkers and Signaling Pathways Implicated in the Pathogenesis of Idiopathic Multicentric Castleman Disease/Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis, Renal Insufficiency, and Organomegaly (TAFRO) Syndrome.

Biomedicines
2024

Dysmorphic megakaryocytes in TAFRO syndrome: A case series from a single institute.

Blood cells, molecules &amp; diseases
2024

A case of TAFRO syndrome after vaccination, successfully treated with cyclosporine.

BMC nephrology
2024

Unraveling TAFRO Syndrome: An In-Depth Look at the Pathophysiology, Management, and Future Perspectives.

Biomedicines
2024

Tacrolimus Treatment for TAFRO Syndrome.

Biomedicines
2024

TAFRO Syndrome on 18F-FDG-PET/CT: An Appealing Diagnostic Tool.

Diagnostics (Basel, Switzerland)
2024

A Case of Thrombocytopenia, Anasarca (Edema, Pleural Effusion, and Ascites), Fever, Reticulin Fibrosis/Renal Dysfunction, and Organomegaly (TAFRO) Syndrome Initially Not Presenting With Thrombocytopenia: A Role of Immature Platelet Fraction.

Cureus
2024

Differentiation Between Disseminated Carcinomatosis of the Bone Marrow From Urothelial Cancer and Intravascular Large B-cell Lymphoma: A Case Report.

Cureus
2024

[Improvement of tissue fibrosis in TAFRO syndrome by prednisolone monotherapy].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2024

Investigation of the Effect of Therapeutic Plasma Exchange for TAFRO Syndrome: A Pilot Study.

Biomedicines
2024

Radiological Imaging Findings of Adrenal Abnormalities in TAFRO Syndrome: A Systematic Review.

Biomedicines
2024

TAFRO Syndrome: A Syndrome or a Subtype of Multicentric Castleman Disease?

Biomedicines
2024

Potential risks of cell-free concentrated ascites reinfusion therapy in TAFRO syndrome patient.

Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy
2024

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

Scientific reports
2024

Successful treatment of streptococcal toxic shock syndrome complicated by primary peritonitis and bilateral empyema in a healthy young woman: Identification of uncommon clone emm103 and novel sequence type 1363.

IDCases
2024

Peritoneal dialysis is feasible for TAFRO syndrome with acute kidney injury and refractory ascites: A case report.

Clinical nephrology
2023

A Case of TAFRO Syndrome Developed after COVID-19 Vaccination.

Case reports in nephrology
2023

TAFRO syndrome in an adolescent with acute hepatitis-like imaging findings.

Pediatrics international : official journal of the Japan Pediatric Society
2023

[Clinical Anslysis of TAFRO Syndrome].

Zhongguo shi yan xue ye xue za zhi
2023

Stepwise Treatment for TAFRO Syndrome.

Journal of medical cases
2023

Case Report: A case of TAFRO syndrome with severe and prolonged thrombocytopenia: diagnostic pitfalls.

Frontiers in immunology
2023

Case report: A case of acute exacerbation of interstitial pneumonia associated with TAFRO syndrome.

Frontiers in medicine
2023

The experience of diagnosis and treatment for TAFRO syndrome.

Annals of hematology
2023

Thrombocytopenia, Anasarca, Myelofibrosis, Renal dysfunction, and Organomegaly (TAFRO) Syndrome Initially Diagnosed as Fibromyalgia: A Case Report.

Cureus
2023

Case report: Rehabilitation course in thrombocytopenia, anasarca, fever, reticulin fibrosis/renal failure, and organomegaly syndrome complicated by cerebral infarction in the left parabolic coronary region.

Frontiers in neurology
2023

Rituximab-bortezomib-dexamethasone induce high response rates in iMCD in clinical practice.

British journal of haematology
2023

Autopsy-proven anaplastic large cell lymphoma mimicking TAFRO syndrome.

Pediatrics international : official journal of the Japan Pediatric Society
2023

A case of TAFRO syndrome with DIC and neurologic and cardiac involvement.

Clinical case reports
2023

[Successful rituximab treatment of TAFRO syndrome refractory to glucocorticoids and tocilizumab].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2023

Combined B-cell immunomodulation with rituximab and belimumab in severe, refractory TAFRO syndrome associated with Sjögren's syndrome: A case report.

Modern rheumatology case reports
2023

Severe systemic inflammation mimicking TAFRO syndrome following COVID-19.

International journal of hematology
2023

Development of rapidly fatal TAFRO syndrome-like features in a patient with essential thrombocythemia.

Journal of clinical and experimental hematopathology : JCEH
2023

Epstein-Barr Virus-Positive Cutaneous and Systemic Plasmacytosis with TAFRO Syndrome-like Symptoms Successfully Treated with Rituximab.

Medicina (Kaunas, Lithuania)
2023

Thrombocytopenia, anasarca, and renal insufficiency as severe and rare complications of Hodgkin lymphoma: a case report.

Journal of medical case reports
2023

TAFRO syndrome was effectively treated with an inexpensive novel scheme: A case report.

Clinical case reports
2023

Evaluation of the correlation between multiple organ calcification on CT and disease severity in patients with TAFRO syndrome.

Japanese journal of radiology
2023

Severe TAFRO Syndrome Mimicking Hepatorenal Syndrome Successfully Treated with a Multidisciplinary Approach: A Case Report and Literature Review.

Internal medicine (Tokyo, Japan)
2023

Siltuximab Monotherapy in Tafro Syndrome: A Case Report and Review of the Literature.

Journal of nephrology
2023

Adolescent-onset TAFRO Syndrome with Malignant Nephrosclerosis-like Lesions.

Internal medicine (Tokyo, Japan)
2022

Treatment-resistant idiopathic multicentric Castleman disease with thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly managed with Janus kinase inhibitors: A case report.

Medicine
2022

Idiopathic Multicentric Castleman Disease Occurring Shortly after mRNA SARS-CoV-2 Vaccine.

Vaccines
2023

TAFRO syndrome: A disease that known is half cured.

Hematological oncology
2023

Acute Abdomen and Adrenal Swelling as the First Manifestations of TAFRO Syndrome.

Internal medicine (Tokyo, Japan)
2022

Global public awareness of Castleman disease and TAFRO syndrome between 2015 and 2021: A Google Trends analysis.

EJHaem
2022

Severe coronavirus disease 2019 in a patient with TAFRO syndrome: A case report.

Clinical infection in practice
2022

TAFRO syndrome with renal biopsy successfully treated with steroids and cyclosporine: a case report.

BMC nephrology
2022

TAFRO syndrome: A severe manifestation of Sjogren's syndrome? A systematic review.

Autoimmunity reviews
2022

Idiopathic plasmacytic lymphadenopathy: A conceptual history along with a translation of the original Japanese article published in 1980.

Journal of clinical and experimental hematopathology : JCEH
2022

Castleman disease, TAFRO syndrome, idiopathic plasmacytic lymphadenopathy, and autoimmune disease.

Journal of clinical and experimental hematopathology : JCEH
2022

A Rare Variant of Idiopathic Multicentric Castleman Disease: TAFRO Syndrome.

Turkish journal of haematology : official journal of Turkish Society of Haematology
2022

TAFRO Syndrome Treated Effectively with Corticosteroids: A Case Report and Review of the Literature.

Indian journal of nephrology
2023

TAFRO Syndrome: A Disease Requiring Immediate Medical Attention.

Internal medicine (Tokyo, Japan)
2022

Successful combination treatment with rituximab, steroid pulse therapy, plasma exchange and romiplostim for very severe TAFRO syndrome.

Leukemia &amp; lymphoma
2023

Mimic or coincidentally? TAFRO syndrome and systemic lupus erythematosus: A case-based review.

Modern rheumatology case reports
2022

An autopsy case of TAFRO syndrome with multiple cerebral infarctions caused by small vessel pathology.

eNeurologicalSci
2022

Multisystem Inflammatory Syndrome in Children: A Case Report From Japan.

Cureus
2022

Post-transplant Complication With TAFRO Features in a Patient With Acute Myeloid Leukemia.

Cureus
2022

International definition of iMCD-TAFRO: future perspectives.

Journal of clinical and experimental hematopathology : JCEH
2022

Historical and pathological overview of Castleman disease.

Journal of clinical and experimental hematopathology : JCEH
2022

TAFRO syndrome with a fatal clinical course following BNT162b2 mRNA (Pfizer-BioNTech) COVID-19 vaccination: A case report.

Journal of infection and chemotherapy : official journal of the Japan Society of Chemotherapy
2022

TAFRO Syndrome That Responded to Prednisolone-only Treatment: Evaluating Changes in IL-6.

Internal medicine (Tokyo, Japan)
2022

Abnormal Exacerbation of Moderately Differentiated Gastric Adenocarcinoma in a Patient with TAFRO Syndrome: An Impaired Tumor Immunity?

Case reports in oncology
2022

Castleman disease and TAFRO syndrome: To improve the diagnostic consciousness is the key.

World journal of clinical cases
2022

A Case of Oral Glomeruloid Hemangioma Without Systemic Conditions.

Cureus
2023

A case of TAFRO syndrome maintained in remission for 5 years after discontinuation of tocilizumab.

Modern rheumatology case reports
2022

Glomeruloid Hemangioma in a Patient with TAFRO Syndrome.

Internal medicine (Tokyo, Japan)
2022

Castleman disease and TAFRO syndrome.

Annals of hematology
2022

Anti-interferon-γ Antibody-seropositive Disseminated Nontuberculous Mycobacterial Infection Mimicking POEMS and TAFRO Syndromes.

Internal medicine (Tokyo, Japan)
2023

TAFRO syndrome with fatigue and abdominal distension as the first symptom: A case report.

Hematological oncology
2022

To and TAFRO - a cryptic cause of acute renal failure: a case report.

BMC nephrology
2021

Successful Treatment of Thrombocytopenia, Anasarca, Fever, Reticulin Myelofibrosis/Renal Insufficiency, and Organomegaly Syndrome Using Plasma Exchange Followed by Rituximab in the Intensive Care Unit.

Journal of medical cases
2022

Renal histological continuum of TAFRO syndrome: A case report and literature review.

Clinical nephrology
2021

Multicentric Castleman disease and the evolution of the concept.

Pathologica
2021

TAFRO Syndrome and Elusive Diagnosis of Idiopathic Multicentric Castleman Disease Treated with Empiric Anti-Interleukin-6 Therapy.

Case reports in oncology
2021

TAFRO Syndrome in a Kidney Transplant Recipient That Was Diagnosed on Autopsy: A Case Report.

Frontiers in medicine
2022

Characteristics of thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly syndrome: a retrospective study from a large Western cohort.

British journal of haematology
2021

Intracranial Hemorrhage in a Patient with TAFRO Syndrome Treated with Cyclosporine A and Rituximab.

Medicina (Kaunas, Lithuania)
2021

A Case of TAFRO Syndrome With Pulmonary Abnormalities.

Journal of thoracic imaging
2021

TAFRO syndrome complicated by porto-sinusoidal vascular liver disease with portal hypertension: a case report.

Clinical journal of gastroenterology
2020

A Patient With Human Herpesvirus 8-Positive Multicentric Castleman's Disease Who Met Criteria for TAFRO Syndrome: Controversy in Practice?

Journal of medical cases
2021

[A case report of TAFRO syndrome].

Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences
2022

Bilateral Adrenal Infarctions as an Initial Manifestation of TAFRO Syndrome: A Case Report and Review of the Literature.

Internal medicine (Tokyo, Japan)
2021

Validated international definition of the thrombocytopenia, anasarca, fever, reticulin fibrosis, renal insufficiency, and organomegaly clinical subtype (TAFRO) of idiopathic multicentric Castleman disease.

American journal of hematology
2021

TAFRO Syndrome with Renal Thrombotic Microangiopathy: Insights into the Molecular Mechanism and Treatment Opportunities.

International journal of molecular sciences
2021

Fatal case of TAFRO syndrome with unilateral adrenal hemorrhage in early-stage disease.

Journal of clinical and experimental hematopathology : JCEH
2021

TAFRO syndrome presenting as intrahepatic cholangitis on autopsy.

Clinical case reports
2021

Patient's age and D-dimer levels predict the prognosis in patients with TAFRO syndrome.

International journal of hematology
2021

TAFRO Syndrome With Kidney Involvement: A Case Series of Patients With Kidney Biopsies.

Kidney medicine
2021

A Review of Genetic Abnormalities in Unicentric and Multicentric Castleman Disease.

Biology
2021

Treating life-threatening TAFRO syndrome with interleukin-1 inhibition.

European journal of internal medicine
2020

A Case of Repeated TAFRO Syndrome-Like Symptoms and Retroperitoneal Hemorrhage in a Patient With Sjögren Syndrome.

Cureus
2020

The Dangers of Immediately Performing a Sensitive and Specific Investigation versus the Dangers of Delaying It: A Cautionary Case of Hodgkin's Lymphoma.

European journal of case reports in internal medicine
2020

[Treatment of TAFRO syndrome with tocilizumab: a case report].

Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi
2021

JAK1/2 Inhibition in Severe TAFRO Syndrome: A Case Report.

Annals of internal medicine
2021

A severe case of thrombocytopenia, anasarca, fever, renal insufficiency or reticulin fibrosis, and organomegaly syndrome with myocardial and skeletal muscle calcification despite hypocalcemia: a case report.

Journal of medical case reports
2021

Borderline Case of TAFRO Syndrome and POEMS Syndrome.

Internal medicine (Tokyo, Japan)
2021

Sequential change in serum VEGF levels in a case of tocilizumab-resistant TAFRO syndrome treated effectively with rituximab.

Modern rheumatology case reports
2020

Kidney biopsy findings in two patients with TAFRO syndrome: case presentations and review of the literature.

BMC nephrology
2021

[TAFRO syndrome and cutaneous necrotizing vasculitis].

La Revue de medecine interne
2021

Complete Resolution of a Case of TAFRO Syndrome Accompanied by Mediastinal Panniculitis, Adrenal Lesion, and Liver Damage with Hyperbilirubinemia.

Internal medicine (Tokyo, Japan)
2020

[HHV-8-positive Castleman's disease with rapidly progressing multiorgan failure mimicking TAFRO syndrome].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2020

Recovery from prolonged thrombocytopenia in patients with TAFRO syndrome: case series and literature review.

Modern rheumatology case reports
2020

Development of severe thrombocytopenia with TAFRO syndrome-like features in a patient with rheumatoid arthritis treated with a Janus kinase inhibitor: A case report.

Medicine
2020

An Autopsy Case of TAFRO Syndrome with Type II Respiratory Failure.

The American journal of case reports
2020

Primary Sjögren's Syndrome Accompanied by Clinical Features of TAFRO Syndrome.

Case reports in rheumatology
2021

Optimal treatments for TAFRO syndrome: a retrospective surveillance study in Japan.

International journal of hematology
2020

Tocilizumab for the treatment of TAFRO syndrome: a systematic literature review.

Annals of hematology
2020

The Clinical and Histopathological Feature of Renal Manifestation of TAFRO Syndrome.

Kidney international reports
2020

Disseminated Mycobacterium genavense infection mimicking TAFRO syndrome.

Journal of infection and chemotherapy : official journal of the Japan Society of Chemotherapy
2020

Ascites in the "TAFRO" Syndrome: Does the Squeeze Make the Juice?

Digestive diseases and sciences
2020

Unexplained cause of thrombocytopenia, fever, anasarca and hypothyroidism: TAFRO syndrome with thrombotic microangiopathy renal histology.

BMJ case reports
2020

A pediatric case of tocilizumab-resistant TAFRO syndrome treated successfully with rituximab.

Pediatric blood &amp; cancer
2020

Frontline Siltuximab and Rituximab in TAFRO syndrome: A case report.

European journal of haematology
2020

Acute Kidney Injury Caused by TAFRO Syndrome in a Chinese Patient: Efficacy of Long-Term Corticosteroids Combined with Bortezomib and Cyclophosphamide.

Kidney &amp; blood pressure research
2020

Computed tomography findings of early-stage TAFRO syndrome and associated adrenal abnormalities.

European radiology
2020

Liver cirrhosis in a child associated with Castleman's disease: A case report.

World journal of clinical cases
2020

Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis/Renal Failure, and Organomegaly (TAFRO) Syndrome with Bilateral Adrenal Hemorrhage in Two Caucasian Patients.

The American journal of case reports
2020

[A report of six TAFRO syndrome: clinical characteristics, diagnosis and treatment analysis].

Zhonghua yi xue za zhi
2020

Genetic basis for iMCD-TAFRO.

Oncogene
2020

Clinical features and treatment of 7 Chinese TAFRO syndromes from 96 de novo Castleman diseases: a 10-year retrospective study.

Journal of cancer research and clinical oncology
2019

Multicentric Castleman disease with TAFRO syndrome and Sjögren's.

Clinical case reports
2020

[TAFRO syndrome, variant of Castleman's disease: case report].

Revista medica del Instituto Mexicano del Seguro Social
2020

TAFRO Syndrome Presenting with Retroperitoneal Panniculitis-like Computed Tomography Findings at Disease Onset.

Internal medicine (Tokyo, Japan)
2020

2019 Updated diagnostic criteria and disease severity classification for TAFRO syndrome.

International journal of hematology
2019

Epidemiological analysis of multicentric and unicentric Castleman disease and TAFRO syndrome in Japan.

Journal of clinical and experimental hematopathology : JCEH
2019

Hepatic Campylobacter jejuni infection in patients with Castleman-Kojima disease (idiopathic multicentric Castleman disease with thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome).

Pathology international
2019

TAFRO syndrome as a cause of glomerular microangiopathy: a case report and literature review.

BMC nephrology
2019

Fatal case of TAFRO syndrome associated with over-immunosuppression: a case report and review of the literature.

Nagoya journal of medical science
2019

TAFRO Syndrome: A Case of Significant Endocrinopathy in a Caucasian Patient.

Cureus
2019

The potential role of follicular helper T cells in idiopathic multicentric Castleman disease with and without TAFRO syndrome.

Pathology, research and practice
2019

Renal Pathologic Findings in TAFRO Syndrome: Is There a Continuum Between Thrombotic Microangiopathy and Membranoproliferative Glomerulonephritis? A Case Report and Literature Review.

Frontiers in immunology
2019

Is TAFRO syndrome a subtype of idiopathic multicentric Castleman disease?

American journal of hematology
2020

Comparison of the clinical characteristics of TAFRO syndrome and idiopathic multicentric Castleman disease in general internal medicine: a 6-year retrospective study.

Internal medicine journal
2019

Subclinical Hypothyroidism in TAFRO Syndrome.

Internal medicine (Tokyo, Japan)
2019

Pathological findings of progressive renal involvement in a patient with TAFRO syndrome.

CEN case reports
2019

A case of cerebral infarction in a patient with TAFRO syndrome.

Journal of the neurological sciences
2019

TAFRO Syndrome with an Anterior Mediastinal Mass and Lethal Autoantibody-Mediated Thrombocytopenia: An Autopsy Case Report.

Acta haematologica
2018

TAFRO Syndrome: A Case Report from Turkey and Review of the Literature.

International journal of hematology-oncology and stem cell research
2019

Clinicopathological features of TAFRO syndrome complicated by acquired hemophilia A and development of cardiopulmonary arrest that were successfully treated with VA-ECMO and tocilizumab.

International journal of hematology
Ver todos os 250 no EuropePMC

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Doenças relacionadas

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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.

  1. Recovery trajectories of TAFRO syndrome: a single-center case series highlighting prolonged thrombocytopenia and anasarca.
    Immunological medicine· 2026· PMID 41866960mais citado
  2. The value of 18F-fluorodeoxyglucose positron emission tomography/computed tomography in the diagnosis and evaluation of TAFRO syndrome: a review of cases and literature.
    Clinical radiology· 2026· PMID 41740223mais citado
  3. Concurrent Idiopathic Multicentric Castleman Disease with TAFRO and Sj&#xf6;gren's Syndrome in a Young Patient: A Challenging Diagnostic Overlap.
    European journal of case reports in internal medicine· 2026· PMID 41668823mais citado
  4. Correction: TAFRO syndrome requiring combined IL 6 and IL 1 inhibition: a case report.
    Frontiers in immunology· 2026· PMID 41659873mais citado
  5. Ruxolitinib in relapsed/refractory TAFRO syndrome: a report of two cases, including one positive for Epstein-Barr virus, and a literature review.
    International journal of hematology· 2026· PMID 41549210mais citado
  6. Successful Treatment of Refractory Pediatric TAFRO Syndrome With Combination Chemotherapy: A Case Report.
    Pediatr Int· 2026· PMID 41983626recente
  7. Ultrasound-guided lateral kidney biopsy in patients unsuitable for the prone position.
    Clin Kidney J· 2026· PMID 41982248recente
  8. Morphological characteristics of the renal lesion in TAFRO syndrome and POEMS syndrome: a retrospective and multicenter study.
    Clin Exp Nephrol· 2026· PMID 41975025recente
  9. [The 516th case: hyperinflammatory state, acute renal dysfunction, hepatosplenomegaly with lymphadenopathy, and recurrent cerebral infarctions].
    Zhonghua Nei Ke Za Zhi· 2026· PMID 41942330recente
  10. Primary adrenal insufficiency and autoimmune thyroiditis in an adolescent with Castleman-TAFRO syndrome.
    JCEM Case Rep· 2026· PMID 41928868recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:457077(Orphanet)
  2. MONDO:0018702(MONDO)
  3. GARD:21902(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q55788267(Wikidata)

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

Síndrome TAFRO
Compêndio · Raras BR

Síndrome TAFRO

ORPHA:457077 · MONDO:0018702
Prevalência
<1 / 1 000 000
Casos
28 casos conhecidos
Herança
Not applicable
CID-10
M35.8 · Outro comprometimento sistêmico especificado do tecido conjuntivo
CID-11
Início
Adolescent, Adult, Elderly
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C4552543
EuropePMC
Wikidata
Papers 10a
Evidência
🥉 Relato de caso
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