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Doença Castleman
ORPHA:160CID-10 · D47.7CID-11 · 4B2YDOENÇA RARA
Esta doença tem causa genética. Recomendamos procurar um médico geneticista para diagnóstico, exames genéticos e aconselhamento familiar.
Sangue / imuneInício todas idadesHerança Unknown
Também conhecida comoHamartoma linfóideHiperplasia ganglionar angiofolicularLinfoma benignoDC
Sinônimos clínicos: Hamartoma linfóide · Hiperplasia ganglionar angiofolicular · Linfoma benigno

A Doença de Castleman (DC) é uma condição benigna (que não é câncer) em que as células de defesa do corpo (chamadas linfócitos) se multiplicam mais do que o normal. Ela pode se apresentar de forma localizada (em apenas um lugar) ou multicêntrica (em várias partes do corpo). Os sintomas são muito variados, indo desde o aumento discreto dos gânglios linfáticos (ínguas) sem causar sintomas, até crises repetidas de inchaço generalizado dos gânglios com sintomas graves que afetam o corpo todo.

Em construçãohistóricoSugerir correção
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Introdução

O que você precisa saber de cara

💬
EM LINGUAGEM SIMPLES

A Doença de Castleman é uma condição não cancerosa em que as células de defesa do organismo se multiplicam mais do que deveriam nos gânglios linfáticos. Ela pode se manifestar em apenas uma região do corpo ou de forma disseminada, provocando ínguas aumentadas, cansaço acentuado, perda de peso e alterações inflamatórias no sangue. No Brasil, a condição ainda não conta com um protocolo clínico e diretriz terapêutica (PCDT) próprio nem remédios específicos cadastrados para entrega oficial pelo SUS. O atendimento ocorre por meio dos serviços especializados de saúde, enquanto pesquisas clínicas internacionais continuam avaliando novos caminhos de tratamento.

📋
Informacoes curadas por IA — podem conter imprecisoes

A Doença de Castleman (DC) é uma condição benigna (que não é câncer) em que as células de defesa do corpo (chamadas linfócitos) se multiplicam mais do que o normal. Ela pode se apresentar de forma localizada (em apenas um lugar) ou multicêntrica (em várias partes do corpo). Os sintomas são muito variados, indo desde o aumento discreto dos gânglios linfáticos (ínguas) sem causar sintomas, até crises repetidas de inchaço generalizado dos gânglios com sintomas graves que afetam o corpo todo.

Pesquisas ativas
13 ensaios
29 total registrados no ClinicalTrials.gov
Publicações científicas
2.035 artigos
Último publicado: 2026 Apr 13

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
All ages
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: D47.7
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

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

Partes do corpo afetadas

🫃
Digestivo
9 sintomas
🩸
Sangue
7 sintomas
🫁
Pulmão
3 sintomas
🫘
Rins
3 sintomas
❤️
Coração
2 sintomas
🛡️
Imunológico
2 sintomas

+ 21 sintomas em outras categorias

Características mais comuns

90%prev.
Linfadenopatia
Muito frequente (99-80%)
55%prev.
Aumento da concentração circulante de interleucina 6
Frequente (79-30%)
55%prev.
Anemia
Frequente (79-30%)
55%prev.
Linfadenopatia mediastinal
Frequente (79-30%)
55%prev.
Dor abdominal
Frequente (79-30%)
55%prev.
Taxa de sedimentação de eritrócitos elevada
Frequente (79-30%)
49sintomas
Muito frequente (1)
Frequente (10)
Ocasional (9)
Muito raro (9)
Sem dados (20)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 49 características clínicas mais associadas, ordenadas por frequência.

LinfadenopatiaLymphadenopathy
Muito frequente (99-80%)90%
Aumento da concentração circulante de interleucina 6Increased circulating interleukin 6 concentration
Frequente (79-30%)55%
Frequente (79-30%)55%
Linfadenopatia mediastinalMediastinal lymphadenopathy
Frequente (79-30%)55%
Dor abdominalAbdominal pain
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico2.035PubMed
Últimos 10 anos200publicações
Pico2025135 papers
Linha do tempo
2026Hoje · 2026🧪 2003Primeiro ensaio clínico📈 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

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
✓Aprovado1
2Fase 29
1Fase 15
·Pré-clínico14
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 29 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Doença Castleman

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

Onde estão os ensaios

Com ensaio aberto em 24 países. O ponto verde marca onde há vaga agora.

🟢 Recrutando agora

10 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

29 ensaios clínicos encontrados, 13 ativos.

Distribuição por fase
NCT06643091 · Nintedanib Treatment in Unicentric Castleman DiseaseEm breve
PHASE2
NCT04742387 · Assessment of Bone Mineral Density in People Living With HIV…Ativo
🇺🇸 Estados Unidos
NCT03933904 · Sirolimus in Previously Treated Idiopathic Multicentric Cast…Ativo
PHASE2🇺🇸 Estados Unidos
NCT04585893 · Safety and Efficacy of Rituximab for Treatment of Multicentr…Concluído
PHASE2Malawi
NCT02853968 · Unlock the Cell: Castleman's Disease Flow Cytometry StudyConcluído
🇺🇸 Estados Unidos
NCT01441063 · Tocilizumab for KSHV-Associated Multicentric Castleman Disea…Concluído
PHASE2🇺🇸 Estados Unidos
NCT05440305 · Unicentric Form of Castleman Disease - Surgery Therapy Benef…Concluído
NCT01183598 · A Study to Provide RoActemra/Actemra (Tocilzumab) to Patient…Concluído
PHASE1🇺🇸 Estados Unidos
NCT00412321 · A Safety and Efficacy Study of CNTO 328 in Patients With B-C…Concluído
PHASE1🇺🇸 Estados Unidos
NCT05081141 · HHV8 and Solid Organ TransplantationUNKNOWN
🇮🇹 Itália
NCT03043105 · TCP Regimen in Newly Diagnosed MCD:a Prospective, Single-cen…UNKNOWN
PHASE2🇨🇳 China
NCT00891280 · Dose-escalation Study of Oral CX-4945UNKNOWN
PHASE1🇺🇸 Estados Unidos
NCT05028621 · Molecular Basis of Langerhans and Non-Langerhans Cell Histio…SUSPENDED
NA🇺🇸 Estados Unidos
NCT03864419 · Rituximab Hyaluronidase in Combination With Chemotherapy in …Encerrado
PHASE1Uganda
NCT02080416 · Nelfinavir for the Treatment of Gammaherpesvirus-Related Tum…Encerrado
EARLY_PHASE1🇺🇸 Estados Unidos
NCT00127569 · Rituximab in the Treatment of HIV Associated Multicentric Ca…Encerrado
PHASE2
NCT02871050 · Castleman Disease Collaborative Network BiobankCancelado
🇺🇸 Estados Unidos
NCT02228512 · Study of Pomalidomide Combined With Modified DA-EPOCH and Ri…Cancelado
PHASE1, PHASE2
NCT00361933 · Valganciclovir to Treat HHV-8 Associated Multicentric Castle…Cancelado
PHASE4
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
1.406 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 1.406

#1

Mesenteric unicentric Castleman's disease misdiagnosed as gastrointestinal neuroendocrine tumour.

BMJ case reports2026 Feb 19

Castleman's disease is a rare lymphoproliferative disorder with a variable presentation ranging from no clinical symptoms to fever and fatigue. We report a case of a male in his 30s who presented to the emergency department with non-specific symptoms, including fever, cough, diarrhoea, abdominal pain and findings of bloody mucus in his stool. Preoperative imaging using CT showed a hypervascular mass in the abdomen measuring 3.9 cm. Positron emission tomography with Gallium-68 DOTA-Tyr3-octreotate (DOTATATE) showed moderate radiotracer uptake, raising suspicion for a neuroendocrine tumour. Preoperative endoscopic ultrasound biopsy results showed a benign reactive lymph node. The patient's case was discussed at a multidisciplinary tumour board, where diagnostic/therapeutic resection was advised. Following resection of the mass, it was found to be an enlarged lymph node with histopathological features consistent with hyaline vascular Castleman's disease. The patient did not experience any complications, postoperative imaging was unremarkable, and he is being monitored closely for recurrence.

#2

Snapshot Look at Castleman Disease.

Journal of cellular and molecular medicine2026 Feb

Castleman disease (CD) is a rare and heterogeneous group of lymphoproliferative disorders characterised by abnormal proliferation of lymphoid tissue. First described in the 1950s, it has since been classified into two major clinical forms: unicentric CD (UCD), involving a single lymph node region and multicentric CD (MCD), which affects multiple regions and is often systemic. Further subclassification of MCD includes HHV8-associated MCD, POEMS-associated MCD and idiopathic MCD (iMCD), each with distinct pathophysiologic mechanisms and clinical implications. This review summarises current understanding of the epidemiology, clinical presentation, histopathology, pathogenesis and diagnostic challenges of CD. It also explores recent advances in molecular biology, including the role of interleukin-6 (IL-6), human herpesvirus-8 (HHV8) and aberrant immune signalling in disease progression. Therapeutic strategies vary significantly depending on the subtype and range from surgical resection in UCD to immunotherapy, siltuximab and cytotoxic chemotherapy in MCD. Despite progress, CD remains underdiagnosed and poorly understood, especially in its idiopathic forms. Continued research into its molecular underpinnings and targeted treatments is critical to improving patient outcomes and establishing evidence- based guidelines.

#3

Monoclonal neutralizing antibodies elicited by infection with Kaposi sarcoma-associated herpesvirus reveal critical sites of vulnerability on gH/gL.

PLoS pathogens2026 Jan

Kaposi sarcoma-associated herpesvirus (KSHV) is an oncogenic virus that causes Kaposi sarcoma, primary effusion lymphoma and multicentric Castleman disease. A vaccine that prevents KSHV infection or serves in the treatment of KSHV-related diseases represents a critical unmet need, however, the types of immune responses a vaccine should elicit have not been well defined. The gH/gL glycoprotein complex is an important target of KSHV-neutralizing antibodies, but the epitope specificities targeted by these antibodies remain unknown. Here, we isolated 12 gH/gL-specific monoclonal antibodies (mAbs) from KSHV-infected donors and performed structure/function analyses. These mAbs bind recombinant gH/gL with nanomolar affinities and epitope binning analyses revealed that the mAbs bind to 5 epitope clusters on gH/gL. Seven mAbs were able to neutralize KSHV infection of epithelial cell lines. Two potent neutralizing mAbs mapped to the EphA2 binding site as determined by inhibition of the receptor-ligand interaction and negative stain electron microscopy (nsEM) of the mAb/gH/gL complex. The epitopes of other neutralizing mAbs targeting novel sites of vulnerability were determined by a combination of cryogenic electron microscopy and nsEM. Together, these mAbs help to define the relevant epitope targets for KSHV vaccine design, have utility in understanding the role of antibodies in preventing KSHV infection, enable the development of immunotherapy approaches, and provide valuable tools to understand the molecular details of the KSHV entry process.

#4

Comprehensive analysis of subtype-specific outcomes and management in Castleman disease: a 20-year cohort study.

Blood advances2026 Mar 10

Castleman disease (CD) is a heterogeneous group of lymphoproliferative disorders anatomically classified by distribution (unicentric CD [UCD], oligocentric CD [oligoCD], or multicentric CD [MCD]). Human herpes virus 8-negative MCD is called idiopathic MCD (iMCD), which includes clinical subtypes with varying phenotypes and responses: TAFRO (thrombocytopenia, anasarca, fever, renal dysfunction and/or reticulin fibrosis, and organomegaly), IPL (idiopathic plasmacytic lymphadenopathy), and not otherwise specified. OligoCD has recently emerged as an intermediate form between UCD and MCD, with unclear clinical behavior, and IPL has not been validated in a Western cohort. We retrospectively analyzed 217 patients with CD evaluated at our institution between January 2004 and August 2024. Survival probabilities were compared using log-rank tests. Overall, 57% had UCD, 20% had oligoCD, and 23% had iMCD. Patients with oligoCD and iMCD more frequently exhibited systemic symptoms than those with UCD. Patients with oligoCD and iMCD had significantly shorter event-free survival (EFS) of 8.9 and 2.3 years, respectively, than those with UCD (not reached; P< .001 and P< .02). Among iMCD subtypes, iMCD-IPL demonstrated a longer EFS than iMCD-TAFRO (P = .02), with no deaths during the follow-up periods. The results validate oligoCD and iMCD-IPL as new subtypes in this Western cohort. The survival outcome in oligoCD was intermediate between UCD and iMCD, with only a subset of patients with oligoCD requiring systemic therapy. iMCD-IPL had a favorable survival outcome in this US cohort, similar to what has been reported in non-Western countries. Tailoring treatment strategies to disease subtypes and vigilant monitoring of oligoCD for progression may improve survival outcomes in CD.

#5

Unicentric Castleman Disease as a Sufficient and Reversible Cause of Steroid-Refractory Autoimmune Hemolytic Anemia in a Child.

Pediatric blood &amp; cancer2026 Mar

Publicações recentes

Ver todas no PubMed

📚 EuropePMC1.404 artigos no totalmostrando 193

2026

Angiomatous Subtype of Penile Kaposi Sarcoma With Concurrent HIV-Negative Multicentric Castleman Disease: A Case Report.

International journal of surgical pathology
2026

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

Immunological medicine
2026

Clinical feature analysis of pediatric Castleman disease with renal involvement.

Pediatric nephrology (Berlin, Germany)
2026

Kaposi sarcoma herpesvirus (KSHV) subtypes and impact on outcomes in KSHV-associated diseases.

The Journal of infectious diseases
2026

Interfollicular Plasmacytosis and Hyperplastic Germinal Centers in Idiopathic Multicentric Castleman Disease, Idiopathic Plasmacytic Lymphadenopathy Subtype.

American journal of hematology
2026

Breast-implant associated unicentric castleman disease: a case report.

Current problems in cancer. Case reports
2026

Case Report: Endothelial-targeted bridging therapy for a TTP-like phenotype in fulminant iMCD-TAFRO.

Frontiers in immunology
2026

Limited discriminatory performance of the iMCD-IPI in a Western cohort.

The oncologist
2026

Transformation of Castleman Disease into Angioimmunoblastic T-Cell Lymphoma Complicated by Hemophagocytic Lymphohistiocytosis: A Case Report and Literature Review.

Case reports in oncology
2026

How I diagnose Castleman disease.

American journal of clinical pathology
2026

Spatial and bulk transcriptomics reveal distinct molecular signatures in Kaposi sarcoma with and without other KSHV-associated diseases.

Journal of translational medicine
2026

Kaposi Sarcoma-Associated Herpesvirus Infection and Complications Among Solid Organ Transplant Recipients - United States, January 2021-September 2025.

MMWR. Morbidity and mortality weekly report
2026

Two Cases of Systemic Lupus Erythematosus With Castleman Disease.

Cureus
2026

Siltuximab 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 oncology
2026

Elucidating the grading intricacies of idiopathic multicentric Castleman disease histopathology: a pathologist's perspective.

American journal of clinical pathology
2026

Effectiveness of tocilizumab for IgA vasculitis associated with idiopathic multicentric Castleman's disease: Two cases and literature review.

Modern rheumatology case reports
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

Is "severe" idiopathic multicentric Castleman disease (iMCD)-idiopathic plasmacytic lymphadenopathy (IPL) really severe?

HemaSphere
2026

Severe polyneuropathy in HIV-negative human herpesvirus 8-associated multicentric Castleman disease successfully treated with rituximab and liposomal doxorubicin.

Journal of infection and chemotherapy : official journal of the Japan Society of Chemotherapy
2026

Mesenteric unicentric Castleman's disease misdiagnosed as gastrointestinal neuroendocrine tumour.

BMJ case reports
2026

Long-term safety and efficacy of filgotinib in patients with idiopathic multicentric Castleman disease: 52-week results from a Phase Ib clinical trial.

Modern rheumatology
2026

Mesenteric Castleman disease: Two case reports and review of literature.

World journal of gastrointestinal surgery
2026

Unicentric Castleman Disease of the Mesentery Mimicking a Gastrointestinal Stromal Tumor: A Case Report.

Cureus
2026

Systemic bone destruction as a presentation of idiopathic multicentric Castleman disease.

British journal of haematology
2026

Clinical diagnosis and treatment analysis of 11 cases of unicentric Castleman disease in the retroperitoneum.

Frontiers in oncology
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

Snapshot Look at Castleman Disease.

Journal of cellular and molecular medicine
2026

Retrospective analysis of primary extranodal unicentric Castleman disease: a systematic review.

Frontiers in medicine
2026

Unicentric Castleman Disease Presenting with a Ruptured Pseudoaneurysm within the Tumor: A Case Report.

Surgical case reports
2026

Successful Bilateral-Lung Transplantation With Long Survival Time for Multicentric Castleman Disease With Fatal Pulmonary Involvement.

Transplantation proceedings
2026

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

JAAD case reports
2025

Unicentric Castleman disease with paraneoplastic pemphigus in a young woman: a case report.

Frontiers in medicine
2026

A Rapid Transition From Thrombocytosis to Thrombocytopenia in Idiopathic Multicentric Castleman Disease.

The Journal of dermatology
2026

HHV-8/KSHV in Solid Organ Transplantation: Current Gaps of Knowledge and Future Directions.

Transplant infectious disease : an official journal of the Transplantation Society
2025

Oligocentric Castleman Disease (OligoCD): A Novel Diagnostic Entity in the Spectrum of Castleman Disease.

Cureus
2026

The clinical significance of the Mediterranean fever gene MEFV variants in Castleman disease.

Communications medicine
2025

The rare report of unicentric Castleman disease with concurrent myasthenia gravis and paraneoplastic pemphigus: a case report with a focused review of the literature.

Frontiers in oncology
2025

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

Frontiers in immunology
2026

KSHV and cancer: understanding the oncogenic machinery for next-generation diagnostic tools and therapies.

Archives of microbiology
2026

Unicentric Castleman Disease as a Sufficient and Reversible Cause of Steroid-Refractory Autoimmune Hemolytic Anemia in a Child.

Pediatric blood &amp; cancer
2026

Clinical features and treatment outcomes of POEMS-associated Castleman disease.

British journal of haematology
2025

Polyclonal 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 spolecnosti
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

Hyperenhancing paravertebral mass in an adolescent: A rare presentation of unicentric Castleman disease.

Radiology case reports
2026

Retrospective cohort evaluation of renal involvement in non-HIV castleman disease patients from a single academic center in Beijing, China.

Annals of hematology
2025

[Castleman disease with systemic manifestation: a case report].

The Pan African medical journal
2026

Clinicohistopathological features and outcome of cutaneous and systemic plasmacytosis in 21 Chinese cases: A retrospective study.

Indian journal of dermatology, venereology and leprology
2026

Idiopathic Multicentric Castleman Disease-TAFRO: A Potentially Curable Disease?

American journal of hematology
2026

Monoclonal neutralizing antibodies elicited by infection with Kaposi sarcoma-associated herpesvirus reveal critical sites of vulnerability on gH/gL.

PLoS pathogens
2026

Endoscopic-Assisted Excision of Parotid Castleman Disease in Pediatric Patients: A Case Series of Three Patients and a Literature Review.

The American journal of case reports
2026

EBV+ and Kaposi's Sarcoma Herpesvirus-Associated Multicentric Castleman Disease in a Patient With HIV Infection: A Case Report.

Case reports in infectious diseases
2026

From the archives of MD Anderson Cancer Center: Paraneoplastic autoimmune multiorgan syndrome (PAMS) associated with stroma-rich Castleman disease.

Annals of diagnostic pathology
2025

A 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.

Cureus
2025

Dysregulated lymphocyte localization in idiopathic multicentric Castleman disease.

bioRxiv : the preprint server for biology
2026

Comprehensive analysis of subtype-specific outcomes and management in Castleman disease: a 20-year cohort study.

Blood advances
2025

Idiopathic Multicentric Castleman Disease With Severe Eosinophilia and Diffuse Centrilobular Nodule-A Rare Case Report.

Case reports in hematology
2025

Castleman disease mimicking prostate cancer metastasis: diagnostic implications of [68Ga]Ga-PSMA-11 PET/CT.

Nuclear medicine review. Central &amp; Eastern Europe
2025

Systemic lupus erythematosus combined with Castleman disease: a case report.

Wiener klinische Wochenschrift
2025

Resection of a Giant Middle Mediastinal Tumor Using a Transaortopulmonary Arterial Approach.

Annals of thoracic surgery short reports
2025

Nocardia farcinica as a potential pathogen associated with a clinical subtype of Kikuchi-Fujimoto disease.

Clinical infectious diseases : an official publication of the Infectious Diseases Society of America
2026

Neurological Peculiarities of POEMS Syndrome: Experience From a Brazilian University Center.

Muscle &amp; nerve
2025

A case of Takayasu arteritis with advanced macrovascular lesions due to 6 years from onset to diagnosis.

Pediatrics international : official journal of the Japan Pediatric Society
2026

Radiation Therapy for Benign Diseases and Premalignant Conditions.

Seminars in radiation oncology
2026

Unicentric Castleman disease with paraneoplastic pemphigus and follicular dendritic cell sarcoma: A case report.

Oncology letters
2025

Imaging approach to retroperitoneal vascular tumors: a narrative review.

Abdominal radiology (New York)
2025

Castleman Disease in a Child: A Rare Cause of Persistent Cervical Lymphadenopathy.

Cureus
2026

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

Immunology letters
2025

Follicular Dendritic Cell Sarcoma Complicated With Preeclampsia and Fetal Growth Restriction in Pregnancy.

Case reports in medicine
2025

Oligocentric Castleman disease: clinical characteristics and surgical outcomes from a single-centre retrospective study.

Frontiers in immunology
2025

Siltuximab 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 hematology
2025

Characteristics of rare diseases cases: A summary analysis of hospitalized patients at a hospital in Western China from 2015 to 2023.

Intractable &amp; rare diseases research
2025

KSHV Acquired During Chemsex With Resultant Multicentric Castleman's Disease in a Patient Successfully Treated for HIV.

Journal of medical virology
2025

Clinicopathological features differentiating Castleman Disease from IgG4-related ophthalmic disease, a 10 years retrospective study.

Indian journal of ophthalmology
2025

ORF45-induced Filamin A phosphorylation promotes cell motility and cell-contact dependent viral infection of Kaposi's sarcoma-associated herpesvirus.

PLoS pathogens
2025

Use of tocilizumab to treat IgA nephropathy complicated by idiopathic multicentric Castleman disease: a case report.

BMC nephrology
2025

IgG4 plasma cells in hematopathology: beyond IgG4-related disease.

Journal of hematopathology
2025

Primary hyaline vascular Castleman disease in the kidney: a report and brief literature review.

Journal of hematopathology
2025

A case of KSHV/HHV8-positive large B-cell lymphoma in a background of HIV-negative KSHV/HHV8-positive Multicentric Castleman disease.

Journal of infection and chemotherapy : official journal of the Japan Society of Chemotherapy
2025

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

Case reports in hematology
2025

[A case of unicentric chest wall Castleman disease-associated paraneoplastic autoimmune multiorgan syndrome].

Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseases
2025

Unicentric Castleman disease in intraparotid lymph node: a case report.

Maxillofacial plastic and reconstructive surgery
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

Advances in KSHV Research: Molecular Pathogenesis, Immune Evasion, and Evolving Therapeutic Horizon.

Journal of medical virology
2025

Clinical Presentation, Treatment, and Outcomes of 28 Patients With Castleman Disease: A Retrospective Analysis of an Italian Cohort.

EJHaem
2025

Interleukin-6 in Castleman disease subtypes: look to tissues, not just blood.

Haematologica
2026

Comparative Safety of Tocilizumab and Siltuximab in Castleman Disease: Pharmacovigilance Study.

American journal of hematology
2025

Plasma Cell Predominance in Lichen Planus Pigmentosus: A Rare Case in a Young Female.

Clinical, cosmetic and investigational dermatology
2025

Iptacopan in C5 blockade refractory atypical hemolytic uremic syndrome with associated Castleman's disease: case report.

BMC nephrology
2025

Diagnostic Challenges in HHV-8-Associated Multicentric Castleman Disease in a Patient with Prior Kaposi Sarcoma.

Dermatopathology (Basel, Switzerland)
2025

Diagnostic and therapeutic challenges in hyaline-vascular idiopathic multicentric Castleman disease: A case report.

The Journal of international medical research
2026

Unicentric Castleman disease presenting as a right lung mass in an 18-year-old man.

Thorax
2025

The heterogeneity of thoracic Castleman disease: implications for classification and personalized management.

Annals of hematology
2025

Effectiveness and safety of tocilizumab, an anti-interleukin-6 receptor antibody, in 342 Japanese patients with multicentric Castleman disease: a 3-year, prospective, observational study.

Modern rheumatology
2025

Bronchiolitis Obliterans in Unicentric Castleman Disease Without Paraneoplastic Pemphigus Requiring Bilateral Lung Transplantation.

Cureus
2026

Anterior mediastinal lesions in TAFRO syndrome: potential clinical significance.

Rheumatology (Oxford, England)
2025

Multicentric Castleman disease detected during the evaluation of tongue cancer: A case report.

Oncology letters
2025

Mesenteric Vessel-Preserving Laparoscopic Surgery for Pediatric Unicentric Castleman Disease in the Transverse Mesocolon.

Surgical case reports
2025

Performance of Large Language Models in Diagnosing Rare Hematologic Diseases and the Impact of Their Diagnostic Outputs on Physicians: Combined Retrospective and Prospective Study.

Journal of medical Internet research
2026

From the archives of MD Anderson Cancer Center: Stroma-rich hyaline vascular Castleman disease followed by indolent T-lymphoblastic proliferation and detection of PDGFRB mutation.

Annals of diagnostic pathology
2026

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

Modern rheumatology
2025

Time to redefine HHV-8 MCD and KICS criteria?

Blood advances
2025

A challenging case of angioproliferative Castleman's disease in the mediastinum.

Respiratory medicine case reports
2025

Practical Imaging Approach to Determining the Cause of Nonneoplastic Lymphadenopathy.

Radiographics : a review publication of the Radiological Society of North America, Inc
2025

[Recent advances in TAFRO syndrome and Castleman disease].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2025

Unicentric Castleman disease in the left adrenal region: a case report and literature review.

Frontiers in oncology
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

The sonographic characteristics of unicentric castleman disease - a single-center retrospective study.

Cancer imaging : the official publication of the International Cancer Imaging Society
2026

Unicentric Subcutaneous Hyaline Vascular Castleman Disease With Concurrent Features of Calcifying Fibrous Tumor.

The American Journal of dermatopathology
2025

Isolated Hilar Mass in Unicentric Castleman Disease: Surgery As the Definitive Diagnosis and Treatment.

Cureus
2025

Castleman Disease and Kaposi Sarcoma: A Review of the Literature and a Case Series.

Journal of clinical medicine
2025

Castleman Disease Variant POEMS Syndrome Presenting as Polyradiculoneuropathy in a Child: A Case Report.

Journal of child neurology
2025

ALK-Rearranged Mesenchymal Neoplasm With Hyaline-Vascular Castleman Disease-Like Features: A Case Report.

Pathology international
2025

Ferritin, C-Reactive Protein, and Soluble CD25 Distinguish TAFRO From HLH.

American journal of hematology
2025

Perioperative Efgartigimod treatment for acute myasthenia gravis exacerbation with retroperitoneal mass: a case report.

BMC neurology
2025

Intrapulmonary Castleman disease mimicking locally advanced primary lung cancer on 18F-FDG PET/CT.

Revista espanola de medicina nuclear e imagen molecular
2025

Parotid Castleman's disease: A case report and literature review.

Oral oncology
2025

Castleman disease: a case report of a pediatric incidental case in Saudi Arabia.

Journal of surgical case reports
2026

Unraveling the clinicopathological diversity of Castleman disease: report of three cases.

QJM : monthly journal of the Association of Physicians
2025

Sequence variability of the K1 gene in Kaposi's sarcoma-associated herpesvirus and its role in pathogenesis.

Current opinion in virology
2026

Robotic Resection of Parapharyngeal Follicular Dendritic Cell Sarcoma With Castleman Disease.

The Laryngoscope
2026

Orbital Castleman Disease on 18 F-FDG PET/CT.

Clinical nuclear medicine
2025

Multicentric Castleman disease with splenic presentation: report of two rare cases with focus on histopathological features and review of the literature.

Virchows Archiv : an international journal of pathology
2025

Distinct interleukin-6 production in IPL and TAFRO subtypes of idiopathic multicentric Castleman disease.

Haematologica
2025

[Hyaline vascular Castleman disease with indolent T-lymphoblastic proliferation complicated by paraneoplastic autoimmune multiorgan syndrome: report of a case].

Zhonghua bing li xue za zhi = Chinese journal of pathology
2025

Atypical clinical presentation associated with Castleman disease: a case report and review of the literature.

Frontiers in medicine
2025

Unicentric Castleman disease treated with rituximab before surgery: clinicopathologic findings.

Annals of hematology
2025

HIV-associated multicentric Castleman disease with Kaposi sarcoma: A case report with 120-week follow-up of HHV-8 after remission.

Journal of infection and chemotherapy : official journal of the Japan Society of Chemotherapy
2025

Castleman disease with synchronous primary rectal and prostate cancers: a case report of successful multimodal treatment and 2-year recurrence-free survival.

Frontiers in oncology
2025

Abdominal Unicentric Castleman Disease: A Hepato-Pancreatico-Biliary Frenemy.

Cureus
2025

Pre-existing oncohematological disease in kidney transplant recipients: impact on graft survival, acute rejection, and long-term clinical outcomes.

Frontiers in immunology
2025

The Evolution and Recent Advances in Diagnostic Criteria for Idiopathic Multicentric Castleman Disease.

American journal of hematology
2025

Oncogenic Mechanisms of Kaposi's Sarcoma-Associated Herpesvirus on Cell Metabolism and Cell Transformation.

Journal of medical virology
2025

Unicentric Hyaline-Vascular Castleman Disease Presenting as a Periduodenal Mass: A Rare Retroperitoneal Manifestation.

Cureus
2025

Lung transplantation in a patient with severe bronchiolitis obliterans secondary to Castleman disease.

Respiratory medicine case reports
2025

A patient-derived CABIN1 mutation recapitulates features of idiopathic multicentric Castleman disease in a mouse model.

Blood advances
2026

Successful tocilizumab monotherapy for multicentric Castleman disease complicated by rheumatoid arthritis and Sjögren's disease: a case-based literature review.

Immunological medicine
2025

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

Indian journal of cancer
2025

Giant Coronary Artery Aneurysm in a Patient With Castleman Disease.

JACC. Case reports
2025

Striking effectiveness of siltuximab-based treatment in refractory idiopathic multicentric Castleman disease resembling IgG4-related disease.

Journal of chemotherapy (Florence, Italy)
2025

Characteristics and outcomes of Kaposi sarcoma herpesvirus-associated inflammatory cytokine syndrome.

Blood advances
2025

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

Nephron
2025

Treatment of Idiopathic Multicentric Castleman's Disease With Sequential Thalidomide-Cyclophosphamide-Prednisone After Siltuximab:Report of One Case.

Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae Sinicae
2025

Idiopathic multicentric Castleman disease resembling autoimmune diseases: A case report.

Medicine
2025

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

BMC ophthalmology
2025

Clinical features and management of head and neck castleman disease: a single-center retrospective analysis.

Acta oto-laryngologica
2025

Retroperitoneal unicentric hyaline vascular variant of Castleman's disease: a case report.

Journal of surgical case reports
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

Complications and Management of Idiopathic Multicentric Castleman Disease.

HCA healthcare journal of medicine
2025

Unicentric Castleman disease following POEMS syndrome remission.

Journal of hematopathology
2025

Follicular dendritic cell sarcoma of the stomach in a young male: A rare case report and literature review.

Medicine
2025

[Clinical Characteristic of Castleman Disease with Renal and Orbit Involvement].

Zhongguo shi yan xue ye xue za zhi
2025

Curative Gastrectomy for Advanced Gastric Cancer in a Patient with Idiopathic Multicentric Castleman Disease: A Rare Case Report.

Surgical case reports
2025

Pathologic Findings of Pulmonary Lymphoproliferative Disorders.

Seminars in ultrasound, CT, and MR
2025

Interferon-γ sparks inflammatory fire in Castleman disease.

Blood
2025

Lymphoproliferative Disorders Mimicked by Tuberculosis: A Retrospective Study on Lateral Flow Urine Lipoarabinomannan (LF-ULAM) Limitations.

Cureus
2025

Spatial and single cell mapping of castleman disease reveals key stromal cell types and cytokine pathways.

Nature communications
2025

A case report of Castleman disease variant of POEMS syndrome presenting with prominent polyserositis and renal impairment.

Frontiers in medicine
2025

Surgical approach to posterior mediastinal Castleman´s disease: a case report.

The Pan African medical journal
2025

An unicentric Castleman disease in the hepatopancreatic space.

Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract
2025

International Consensus Histopathological Criteria for Subtyping Idiopathic Multicentric Castleman Disease Based on Machine Learning Analysis.

American journal of hematology
2025

Concurrent Presentation of Human Herpesvirus 8-Associated Multicentric Castleman Disease and Kaposi Sarcoma in a Young Patient.

Cureus
2025

Paraneoplastic Limbic Encephalitis in a Patient With Thymoma.

Cureus
2025

Non-Clonal Lymphoproliferative Diseases.

Hematological oncology
2025

[A case of pulmonary nodules and cysts associated with hypereosinophilia].

Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseases
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

Hemophagocytic lymphohistiocytosis in people living with HIV-a single centre experience.

Infection
2025

18F-FDG PET/CT of a Multicentric Castleman Disease with Lymph Node and Skin Involvement.

Molecular imaging and radionuclide therapy
2026

Expert Perspective: Diagnosis and Treatment of Castleman Disease.

Arthritis &amp; rheumatology (Hoboken, N.J.)
2025

Unicentric Castleman disease: CT and cinematic rendering imaging features.

Diagnostic and interventional imaging
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

Diagnostic pitfall: Castleman's disease versus systemic lupus erythematosus in a woman with generalised lymphadenopathy.

BMJ case reports
2025

[Pulmonary Castleman disease: At the crossroads of thoracic and hematologic pathology].

Annales de pathologie
2025

Kaposi sarcoma and Kaposi inflammatory cytokine syndrome following allogeneic haematopoietic stem cell transplantation: A case report.

British journal of haematology
2025

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

Pediatric blood &amp; cancer
2025

Exploring castleman disease in a cohort of hispanic patients: a recognition to its histopathology.

Journal of hematopathology
2025

Autoantibodies Directed Against Insulin Receptor During the Course of Castleman Disease: A New Case Reaffirming Autoimmune Hypoglycemia as a Relapse Warning Signal.

European journal of case reports in internal medicine
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

[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 hematology
2025

Kaposi Sarcoma-Associated Herpesvirus Risk and Disease in Kidney Donors and Transplant Recipients with HIV in the United States.

Clinical infectious diseases : an official publication of the Infectious Diseases Society of America
2025

Rare Disease Drug Repurposing.

JAMA network open
2025

Rare Unicentric Intrapulmonary Castleman Disease: A Systematic Review and Report of a Case.

The open respiratory medicine journal
2025

Prognostic value of serum C-reactive protein in idiopathic multicentric Castleman disease and construction of a prognostic model for patients.

Frontiers in medicine
2025

Isolated involvement of parotid in unicentric Castleman disease.

BMJ case reports
2025

Reversible cerebral vasoconstriction syndrome in idiopathic multicentric Castleman disease under treatment with tocilizumab.

BMJ neurology open
2025

Laboratory Characterization of Co-Infections in Individuals Infected with HHV-8.

Viruses
2025

Castleman Disease-Still More Questions than Answers: A Case Report and Review of the Literature.

Journal of clinical medicine
2025

The Involvement of PI3K-Akt Signaling in the Clinical and Pathological Findings of Idiopathic Multicentric Castleman Disease-Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis, and Organomegaly and Not Otherwise Specified Subtypes.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2025

Unmasking POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes) Syndrome in a Heart Failure Patient: A Diagnostic Challenge.

Cureus
2025

Efficacy of tocilizumab monotherapy for autoimmune hemolytic anemia associated with idiopathic multicentric Castleman disease: a case-based review.

Rheumatology international
2025

Castleman disease of stomach treated by endoscopic submucosal dissection: a case report and literature review.

Frontiers in oncology
2025

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

Internal medicine (Tokyo, Japan)
2025

Spatial transcriptomics study of Castleman disease.

Journal of translational medicine
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Doença com base genética

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Conselhos e sociedades

Doenças relacionadas

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Perguntas frequentes

O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu

Respostas geradas por IA a partir das fontes citadas

Não, a Doença de Castleman é definida oficialmente como uma proliferação benigna dos linfócitos do sistema imune. Embora possa apresentar comportamento inflamatório intenso e exigir atenção médica constante, não se trata de uma neoplasia maligna.

Referências

Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete

10 publicações do grafo RarasNet (PubMed) · 6 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.

  1. Snapshot Look at Castleman Disease.
    Journal of cellular and molecular medicine2026PMID 41664979
  2. ORPHA:160
    Orphanet
  3. GARD:12656
    GARD (NIH)
  4. Q1947302
    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.

Citar este verbete

Raras. (s.d.). Doença Castleman. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/doenca-castleman

Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Compêndio · Raras BR

Doença Castleman

ORPHA:160 · MONDO:0015564
Prevalência
Unknown
Herança
Not applicable
CID-10
D47.7 · Outras neoplasias especificadas de comportamento incerto ou desconhecido dos tecidos linfático, hematopoético e tecidos relacionados
CID-11
Ensaios
13 ativos
Início
All ages
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0017531
EuropePMC
Wikidata
Wikipedia
Papers 10a

📋 Origem dos dados

Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.

Doença rara (ontologia)
fonte: Orphanet
Identificador unificado
fonte: MONDO
Indexação biomédica
fonte: MeSH (NLM)
Dado público estruturado
fonte: Wikidata
Rara