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Síndrome hemofagocítico associado a infecção
ORPHA:158048CID-10 · D76.2CID-11 · 3B62.5DOENÇA RARA

A Síndrome hemofagocítica, ou Linfo-histiocitose hemofagocítica, é uma condição rara, relacionada à hiperativação imunológica, que desencadeia um estado hiperinflamatório, levando aos sinais e sintomas da síndrome, como febre alta prolongada, hepatoesplenomegalia e citopenias. A HLH pode ter origem genética – HLH familiar ou primária – ou ser secundária a quadros infecciosos, reumatológicos, malignos ou metabólicos – HLH adquirida ou secundária.

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Introdução

O que você precisa saber de cara

📋

Síndrome hemofagocítica associada a infecção é uma condição rara caracterizada por resposta imune desregulada a infecções (como CMV, EBV), levando a inflamação sistêmica, esplenomegalia, hepatomegalia e citopenias. Pode cursar com sintomas neurológicos como ataxia e paralisia de nervos cranianos.

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: D76.2
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Sinais e sintomas

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

Partes do corpo afetadas

🛡️
Imunológico
7 sintomas
🧠
Neurológico
4 sintomas
🩸
Sangue
4 sintomas
🫃
Digestivo
3 sintomas
❤️
Coração
2 sintomas
🦴
Ossos e articulações
1 sintomas

+ 14 sintomas em outras categorias

Características mais comuns

100%prev.
Hemofagocitose
90%prev.
Distribuição anormal de subpopulações de linfócitos T
Muito frequente (99-80%)
90%prev.
Esplenomegalia
Muito frequente (99-80%)
90%prev.
Sinalização anormal de citocinas
Muito frequente (99-80%)
90%prev.
Pancitopenia
Muito frequente (99-80%)
90%prev.
Resposta inflamatória anormal
Muito frequente (99-80%)
35sintomas
Muito frequente (9)
Frequente (12)
Ocasional (11)
Muito raro (3)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 35 características clínicas mais associadas, ordenadas por frequência.

HemofagocitoseHemophagocytosis
Muito frequente100%
Distribuição anormal de subpopulações de linfócitos TAbnormal T cell subset distribution
Muito frequente (99-80%)90%
EsplenomegaliaSplenomegaly
Muito frequente (99-80%)90%
Sinalização anormal de citocinasAbnormal cytokine signaling
Muito frequente (99-80%)90%
PancitopeniaPancytopenia
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Últimos 10 anos200publicações
Pico2025162 papers
Linha do tempo
2026Hoje · 2026🧪 2016Primeiro 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

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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.
3Fase 32
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 2 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Síndrome hemofagocítico associado a infecção

🗺️

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

Pesquisa e ensaios clínicos

0 ensaios clínicos encontrados.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
0 papers (10 anos)
#1

Unraveling the complexities: A comprehensive review of severe fever with thrombocytopenia syndrome and its associated complications.

iScience2026 Apr 17

Severe fever with thrombocytopenia syndrome (SFTS), caused by Dabie bandavirus (DBV), is an emerging vector-borne infection with significant global health implications, particularly in East Asia. In recent years, its geographic footprint has expanded beyond traditional endemic regions, raising global concern due to high morbidity and variable case-fatality rates of up to 30%. Clinically, SFTS presents with an acute onset of high fever, thrombocytopenia, leukocytopenia, headache, and gastrointestinal disturbances. In severe cases, rapid progression may lead to life-threatening complications, including myocarditis, neurological disorders, invasive pulmonary aspergillosis (IPA), secondary infections, systemic inflammatory response syndrome (SIRS), hemophagocytic lymphohistiocytosis (HLH), and ultimately multi-organ failure (MOF). This review synthesizes recent advances in virology, pathophysiological mechanisms, diagnostic strategies, and therapeutic interventions for DBV infections. Special attention is given to the complications most commonly associated with poor prognoses and fatal outcomes in patients with SFTS, alongside future outlooks for research and public health preparedness to mitigate the growing global burden of this high-priority tick-borne disease.

#2

Epstein-Barr virus infection in children with liver transplantation.

World journal of hepatology2026 Feb 27

Epstein-Barr virus (EBV) infection commonly occurs in children, particularly in developing countries. Most children infected with EBV are asymptomatic, though some develop significant complications, including EBV-associated malignancies, hemophagocytic lymphohistiocytosis, and multiple sclerosis. In immunocompromised children, including those with liver transplantation, EBV infection manifests with a diverse spectrum of presentations, varying from asymptomatic to post-transplant lymphoproliferative disorder, which can evolve into lymphoma. Therefore, close monitoring, early detection, and prompt management are crucial. This review aimed to investigate the pathogenesis and manifestations of EBV in healthy children and those who underwent liver transplantation. The proposed algorithm for early EBV detection and management, along with case studies, is provided to help pediatricians increase their index of suspicion.

#3

The features and prognosis analysis of central nervous system Epstein-Barr virus infection in children with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.

European journal of pediatrics2026 Feb 06

The purpose of the study is to analyze the clinical features, treatment, and survival outcomes of Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (EBV-HLH) with positive cerebrospinal fluid (CSF) EBV-DNA in children. Patients diagnosed with EBV-HLH admitted to our center from January 2019 to August 2024 were enrolled and divided into a CSF-EBV group and a non-CSF-EBV group based on CSF EBV-DNA test results. Neurological manifestations were observed in only 33.3% (13/39) of children with CSF-EBV positivity, while 48.7% (19/39) showed abnormal brain magnetic resonance imaging findings. The CSF-EBV group had a higher blood EBV-DNA load (P = 0.002) and a lower CD4+/CD8+ ratio (P = 0.034) compared with the non-CSF-EBV group. A strong positive correlation was observed between CSF EBV-DNA load and CSF cell count (r = 0.800, P < 0.0001). Overall survival was shorter in the CSF-EBV group (P = 0.017). Multivariate Cox regression identified EBV-associated central nervous system (EBV-CNS) infection as a potential independent risk factor for poor prognosis (HR = 6.077, 95% CI: 1.345-27.435, P = 0.019). Intrathecal methotrexate and dexamethasone effectively reduced CSF viral load (P = 0.003) and cell count (P = 0.042), but did not significantly improve overall survival (P = 0.3). EBV-CNS infection may be an independent risk factor for poor prognosis in pediatric EBV-HLH, underscoring the importance of CSF EBV-DNA testing, particularly in patients with high blood loads. Although intrathecal therapy effectively reduced CNS viral load and inflammation, its survival benefit requires further validation in large-scale prospective studies. • CNS involvement is a well-established poor prognostic factor in HLH. • EBV-CNS infection is an independent prognostic risk factor in adult EBV-HLH. • EBV-CNS infection is an independent prognostic risk factor in pediatric EBV-HLH. •While intrathecal therapy effectively reduces CSF viral load and inflammation, its survival benefit still requires further validation through large-scale prospective studies.

#4

Prognostic Value of Laboratory Biomarkers in Risk Stratification for Short-Term Outcomes in Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis.

Current medical science2026 Feb

This study aimed to investigate the association between laboratory biomarkers and short-term poor prognosis in patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) and to develop a risk stratification model. A retrospective analysis was conducted on clinical data from 117 EBV-HLH patients admitted to our hospital between June 2016 and December 2024. Patients were classified into poor prognosis (n = 48) and good prognosis (n = 69) groups based on 28-day outcomes. Potential predictors were screened by univariable logistic regression and receiver operating characteristic (ROC) curve analysis, and a composite laboratory-based risk scoring system was subsequently constructed. The poor prognosis group exhibited significantly higher levels of urea (UREA), direct bilirubin (DB), high-sensitivity cardiac troponin I (hscTnI), serum ferritin (Ferr), and prothrombin time (PT) than the good prognosis group did (all P < 0.05). ROC analysis determined the optimal cutoff values and corresponding odds ratios (ORs) for poor prognosis as follows: UREA (≥ 5.4 mmol/L, OR = 5.911), DB (≥ 10.0 μmol/L, OR = 2.524), hscTnI (≥ 7.4 pg/mL, OR = 2.747), Ferr (≥ 12,422 μg/L, OR = 2.366), and PT (≥ 14.1 s, OR = 3.221). A 0-5-point risk score model was constructed based on these thresholds. The incidence of poor prognosis increased progressively with the score: 23.08% (score 0-1), 27.59% (score 2), 45.00% (score 3), 66.67% (score 4), and 92.30% (score 5). Each 1-point increase in the score was associated with an OR of 1.915 for poor prognosis. The composite risk scoring system incorporating UREA, DB, hscTnI, Ferr, and PT showed satisfactory predictive performance for short-term outcomes in EBV-HLH patients. A score of ≥3 identifies high-risk individuals who may benefit from intensified immunomodulatory therapy, thereby facilitating individualized and stratified clinical management.

#5

Construction of a forest plot prediction model based on Lasso regression for Epstein-Barr virus associated hemophagocytic lymphohistiocytosis in children.

Italian journal of pediatrics2026 Jan 05

To identify risk factors for the progression of Epstein-Barr virus(EBV) infection to Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) and guide clinical intervention by analyzing the clinical data and laboratory examination between infectious mononucleosis and EBV-HLH infection using a forest plot prediction model. Clinical data and laboratory tests of children with “Epstein-Barr virus infection and hemophagocytic lymphohistiocytosis” who were hospitalized in Children’s Hospital of Soochow University from January 2019 to December 2024 were collected. A total of 1358 children of infectious mononucleosis associated with EBV (IM group) and 86 children of hemophagocytic lymphohistiocytosis associated with EBV (EBV-HLH group) were included. The differences between the groups were retrospectively analyzed and regression analysis was performed. The proximity matching method was selected for 1:4 matching between the EBV-HLH group and the IM group. The forest plot prediction model was established based on Lasso regression to analyze the clinical differences between the IM group and the EBV-HLH group. Lasso regression model screening identified hemoglobin (HB), ferritin (FER), fibrinogen (FIB) and CD3 + CD4 + as hexhibiting good predictive value for EBV-HLH, with areas under the receiver operating characteristic (ROC) curve of 0.904, 0.973, 0.866 and 0.783, and specificities of 0.799, 0.965, 0.802 and 0.892, respectively. The prediction model constructed using HB, FER, FIB, and CD3 + CD4 + showed excellent predictive accuracy. With an optimal cut-off value of F = 56.95, the model achieved a sensitivity of 95.30% and a specificity of 99.70%. The early diagnosis of EBV-HLH lacks specific indicators. In this study, a predictive model for EBV-HLH was established using LASSO regression, incorporating four key parameters (HB, FER, FIB, and CD3 + CD4 + T-cell subsets). This model may serve as a screening tool for the early diagnosis of EBV-HLH and provide a diagnostic basis for clinical practice.​ Not applicable.

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The features and prognosis analysis of central nervous system Epstein-Barr virus infection in children with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.

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Unmasking Epstein-Barr Virus (EBV)-Positive Large B-Cell Lymphoma as an Underlying Trigger in a Post-COVID Hemophagocytic Lymphohistiocytosis Case.

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A critically ill adolescent with EBV-associated hemophagocytic lymphohistiocytosis-induced hyperinflammatory shock: a case report and literature review.

Frontiers in immunology
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Prognostic Value of Laboratory Biomarkers in Risk Stratification for Short-Term Outcomes in Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis.

Current medical science
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mNGS facilitates the diagnosis of pediatric murine typhus: A case report.

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Coagulation dysfunction in children with secondary hemophagocytic lymphohistiocytosis: a comprehensive analysis.

Annals of medicine
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[Unusual presentation of Crohn disease associated with hemophagocytic lymphohistiocytosis syndrome: a case report].

Revista de gastroenterologia del Peru : organo oficial de la Sociedad de Gastroenterologia del Peru
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Role of Hemoperfusion With HA330 in the Management of Severe Dengue Shock Syndrome.

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Herpes simplex virus 2-associated symmetrical peripheral gangrene in an immunocompetent fourteen-year-old girl: a case report.

Translational pediatrics
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Prognostic implications of adverse events associated with CAR-T cell therapy: a population-based global observational study.

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Construction of a forest plot prediction model based on Lasso regression for Epstein-Barr virus associated hemophagocytic lymphohistiocytosis in children.

Italian journal of pediatrics
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West Nile Virus-Associated Hemophagocytic Lymphohistiocytosis, Switzerland.

Emerging infectious diseases
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Chronic Active Epstein-Barr Virus Disease: Molecular Pathogenesis, Evolving Concepts, and Emerging Therapies.

Blood
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Epidemiological, clinical characteristics and prognostic factors analysis of adult patients with hemophagocytic lymphohistiocytosis in a Chinese hospital.

Frontiers in immunology
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Pembrolizumab-Associated Hemophagocytic Lymphohistiocytosis in Clear Cell Renal Carcinoma: Case Report and Literature Review.

Reports (MDPI)
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Primary Hemophagocytic Lymphohistiocytosis: A Severe Immune Dysregulatory Disease with Various Genotypic Features and Outcomes: A Cross-Sectional Study from a Tertiary Pediatric Center.

Turkish journal of haematology : official journal of Turkish Society of Haematology
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HHV-8-Associated Hemophagocytic Lymphohistiocytosis in a HIV-Negative and Nontransplant Man: A Case Report and Literature Review.

Case reports in infectious diseases
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A Case of Severe Dengue With Hemophagocytic Lymphohistiocytosis (HLH) Successfully Managed With Tocilizumab and Dexamethasone.

Cureus
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Parvovirus B19-Induced Aplastic Crisis and Hemophagocytic Lymphohistiocytosis in a Child With Hereditary Spherocytosis.

Cureus
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Prevalence and Diagnostic Challenge of Hemophagocytic Lymphohistiocytosis Syndrome in Critically Ill Patients.

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Cytomegalovirus-induced intrahepatic cholestasis complicated by hemophagocytic syndrome in an immunocompetent adult.

Clinical journal of gastroenterology
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Flow cytometric analysis of the immune cell subsets in adult hemophagocytic lymphohistiocytosis.

Frontiers in immunology
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Hemophagocytic Lymphohistiocytosis Associated With Disseminated Histoplasmosis in a Patient With Chronic Pancytopenia: A Rare Case.

Cureus
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HIV infection complicated with talaromyces marneffei, tuberculosis, hemophagocytic lymphohistiocytosis and non-Hodgkin lymphoma: a complex case report.

BMC infectious diseases
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Hemophagocytic lymphohistiocytosis triggered by Hashimoto thyroiditis during pregnancy: a rare case report and diagnostic challenge.

Annals of medicine and surgery (2012)
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Metagenomic Next-generation Sequencing Aids Diagnosis of Leishmania donovani -associated Hemophagocytic Lymphohistiocytosis in Infants: Case Report and Systematic Review.

The Pediatric infectious disease journal
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Hemophagocytic lymphohistiocytosis: do we have a solution for TMI (too much inflammation)?

Hematology. American Society of Hematology. Education Program
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Emapalumab use in malignancy-associated hemophagocytic lymphohistiocytosis in the United States: the REAL-HLH study.

Blood advances
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Hemophagocytic Lymphohistiocytosis in HIV/AIDS: Who Fired the First Shot, the Virus or Its Companions?

Cureus
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Case Report: Blood and cerebrospinal fluid mNGS-assisted diagnosis Toxoplasma gondii infection-associated with hemophagocytic syndrome and systemic lupus erythematosus.

Frontiers in medicine
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Case Report: Conservative management of hemophagocytic lymphohistiocytosis with fulminant hepatic failure in a pediatric leukemia patient.

Frontiers in pediatrics
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Hemophagocytic lymphohistiocytosis as the initial manifestation of Epstein-Barr virus-related T/NK-cell lymphoproliferative disorders in a pediatric patient: a case report and literature review.

Frontiers in pediatrics
2025

Piperacillin-tazobactam-induced hemophagocytic lymphohistiocytosis in a patient with community-acquired pneumonia: A case report and literature review on diagnostic challenges of elevated procalcitonin.

Medicine
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Successful treatment of a dog with a severe Babesia vulpes infection and associated haemophagocytic syndrome after splenectomy.

Topics in companion animal medicine
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Fatal Chronic Varicella-Zoster Viral Infection in a Young Man With Chediak-Higashi Syndrome.

Pediatric dermatology
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Hemophagocytic lymphohistiocytosis in the context of hepatitis-associated severe aplastic anemia: A case report.

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Invasive fusariosis after CD19 chimeric antigen receptor T-cell therapy.

ASM case reports
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Risk-Stratified Phase II Clinical Trial for Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis in Pediatric, Adolescent, and Young Adult Patients in Japan.

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Acute liver failure as a rare presentation of miliary tuberculosis with clinical improvement following prediagnostic corticosteroid therapy: A case report.

International journal of infectious diseases : IJID : official publication of the International Society for Infectious Diseases
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Macrophage Activation-Like Syndrome in critically ill patients with sepsis: high risk patients with high mortality.

Anaesthesia, critical care &amp; pain medicine
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Fever: Harmless Heat or a Deadly Inferno? Unmasking Hemophagocytic Lymphohistiocytosis and Beyond: A Rare Case of Mast Cell Sarcoma and Overview of HLH.

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Histoplasmosis-Associated Hemophagocytic Lymphohistiocytosis in the Setting of Granulomatosis With Polyangiitis.

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Philadelphia Chromosome-Negative Hyperdiploid B-cell Acute Lymphoblastic Leukemia Presenting As Hemophagocytic Lymphohistiocytosis in a Young Adult: A Case Report.

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Hemophagocytic Lymphohistiocytosis (HLH) Induced by Epstein-Barr Virus in a Patient With Human Immunodeficiency Virus (HIV) Infection.

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Monocyte/macrophages may exert prominent roles in macrophage activation syndrome.

Annals of hematology
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[Successful treatment of rapidly progressive Epstein-Barr virus-associated lymphoproliferative disorder with upfront allogeneic hematopoietic cell transplantation].

[Rinsho ketsueki] The Japanese journal of clinical hematology
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Characteristics of Secondary Malignancy Among Children With Primary Immunodeficiency Disorders in Saudi Arabia.

Cureus
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Analysis of risk factors for early death of lymphocyte subsets in adult patients with secondary hemophagocytic lymphohistiocytosis.

Experimental hematology
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Hemophagocytic lymphohistiocytosis in 60 Mexican children with chronic granulomatous disease.

Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology
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Risk and response adapted treatment in pediatric Epstein-Barr virus associated hemophagocytic lymphohistiocytosis.

The Journal of infectious diseases
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Extranodal Epstein-Barr Virus-Positive NK/T Non-Hodgkin Lymphoma With Cutaneous Involvement: A Case Report.

Pediatric dermatology
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Acquired Hypolipoproteinemia and Hemophagocytic Lymphohistiocytosis: A Case Series and Review.

Hematology reports
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When Hepatitis Masks Visceral Leishmaniasis: A Case of Secondary HLH in a Low-Endemic Region.

Clinical case reports
2025

Disseminated mucormycosis leading to a fatal gastrointestinal perforation in a pediatric case of EBV-associated Hemophagocytic lymphohistiocytosis.

Medical mycology case reports
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Rubella-associated granuloma in a patient with a compound heterozygous RAG1 defect and review of the literature.

Immunologic research
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Clinical manifestations and outcomes of EBV-related secondary hemophagocytic lymphohistiocytosis.

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Frontiers in immunology
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Frontiers in immunology
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Clinical characteristics and prognostic factors in adult macrophage activation syndrome: an observational study in a Chinese cohort.

Lupus science &amp; medicine
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International journal of infectious diseases : IJID : official publication of the International Society for Infectious Diseases
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Annals of medicine
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Italian journal of pediatrics
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Secondary Hemophagocytic Lymphohistiocytosis Triggered by Subdural Empyema due to Streptococcus constellatus: A Rare Triad.

Journal of Brown hospital medicine
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Hemophagocytic lymphohistiocytosis: an update in diagnostics, criteria, and treatment considerations.

Current opinion in pediatrics
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Journal of medical case reports
2025

Brucellosis-associated hemophagocytic lymphohistiocytosis: a case report and literature review.

Frontiers in medicine
2025

Two cases of combined immunodeficiency with ITPR3 mutations presenting with life-threatening severe EBV-associated hemophagocytic lymphohistiocytosis.

Frontiers in immunology
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Neonatal Sepsis Caused by Human Parechovirus Type A3 With Marked Hyperferritinemia: A Case Report.

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Journal of personalized medicine
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Case Report: Life-threatening overlap of hemophagocytic syndrome and atypical hemolytic uremic syndrome in a patient with autoimmune polyglandular syndrome type 1 successfully treated with targeted immunotherapy.

Frontiers in immunology
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Hemophagocytic Lymphohistiocytosis in the adult critically ill: a narrative review of case reports and case series.

Frontiers in medicine
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UNC13D c.2588G>A Nucleotide Variant Impairs NK-Cell Cytotoxicity in Adult-Onset EBV-Associated Hemophagocytic Lymphohistiocytosis: A Pedigree Study.

International journal of molecular sciences
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Lymphoid and Myeloid Proliferations After Chimeric Antigen Receptor (CAR) T-Cell Therapy: The Pathologist's Perspective.

International journal of molecular sciences
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[Correlation Analysis of Inflammatory Indexes and Bone Marrow Cytological Characteristics with Prognosis in Patients with Hemophagocytic Lymphohistiocytosis].

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Autopsy Case of a Disseminated Cytomegalovirus Infection with a Cutaneous Ulcer Mimicking Rheumatoid Vasculitis.

Internal medicine (Tokyo, Japan)
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Clinical and genetic features of UNC13D deficiency with hypogammaglobulinemia.

Frontiers in immunology
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Hemophagocytic lymphohistiocytosis in patients with inflammatory bowel diseases: a systematic review.

Frontiers in immunology
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Case Report: Hemophagocytic lymphohistiocytosis associated with NBAS gene variant and Epstein-Barr virus (EBV) infection.

Frontiers in pediatrics
2025

Hemophagocytic Lymphohistiocytosis: An 18-Year-Long Retrospective Single-Center Study.

Cureus
2025

Hemophagocytic Lymphohistiocytosis Gene Variants in Severe COVID-19 Cytokine Storm Syndrome.

Viruses
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Hemophagocytic Lymphohistiocytosis Triggered by Dengue: A Narrative Review and Individual Patient Data Meta-Analysis.

Viruses
2025

Case Report: Uncommon presentation of Ewing sarcoma complicated by hemophagocytic lymphohistiocytosis: a diagnostic dilemma and therapeutic challenge.

Frontiers in oncology
2025

Hemophagocytic syndrome secondary to canine visceral leishmaniasis: Relationship with clinical score, parasite load and serum cytokines.

Comparative immunology, microbiology and infectious diseases
2025

Lactic acidosis in hemophagocytic syndrome: diagnostic and therapeutic challenges in the intensive care unit-a case series.

Journal of medical case reports
2025

Exploring the Complexities of Liver Transplantation for Secondary Hemophagocytic Lymphohistiocytosis-Associated Acute Liver Failure.

Pediatric transplantation
2025

EB virus infection-associated hemophagocytic lymphohistiocytosis complicated with giant axillary lymph nodes: A case report.

Medicine
2025

Progressive Disseminated Histoplasmosis with Dermatomyositis and Macrophage Activation Syndrome/Hemophagocytic Lymphohistiocytosis.

The American journal of tropical medicine and hygiene
2025

Primary Immunodeficiencies and EBV-Associated Diseases.

Current topics in microbiology and immunology
2025

Brucella-associated hemophagocytic syndrome: case report of a potentially life-threatening condition and literature review.

Frontiers in immunology
2025

Epstein-Barr Virus (EBV) Masquerading as Exudative Tonsillitis and Rash in a Transgender Adolescent.

Cureus
2025

Elevated CD4⁺/CD8⁺ ratio and D-dimer as diagnostic biomarkers for Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis versus infectious mononucleosis in children: a retrospective case-control study.

BMC infectious diseases
2025

Polymerase Chain Reaction-Based Diagnosis of Leishmaniasis Targeting Kinetoplast Minicircle DNA in Infants with Hemophagocytic Lymphohistiocytosis.

The American journal of tropical medicine and hygiene
2025

Secondary Dengue Infection Complicated by Hemophagocytic Lymphohistiocytosis: A Case Report.

Case reports in infectious diseases
2025

Genetics of progressive multifocal leukoencephalopathy: update on case reports with an inborn error of immunity and risk variants found in drug-linked cases.

Frontiers in neurology
2025

An Unusual Case of Acute Epstein-Barr Virus Hepatitis Presenting as Severe Cholestatic Liver Disease Inducing Hemophagocytic Lymphohistiocytosis in a Young Adult: A Case Report.

Cureus
2025

Acute Myeloid Leukemia with Hemophagocytosis: Diagnostic Cues and Potential Pitfalls.

Annals of clinical and laboratory science
2025

Biomarkers of pediatric Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis through single-cell transcriptomics.

Nature communications
2025

Expression profiling of Epstein-Barr virus-derived microRNA in systemic chronic active EBV disease.

International journal of hematology
2025

Etiologies and long-term outcome of pediatric hemophagocytic lymphohistiocytosis and macrophage activation syndrome in Taiwan: a single-center retrospective study.

Frontiers in immunology
2025

Secondary Hemophagocytic Lymphocytosis in Inflammatory Bowel Disease.

Hematology reports
2025

Hemophagocytic lymphohistiocytosis syndrome (HLH) in HIV-associated Kaposi sarcoma patients: The first case series from a single center in Türkiye.

International journal of STD &amp; AIDS
2025

Griscelli Syndrome: A Case Report from Pakistan, A Review of the Literature, and an Approach to Hematological Disorders Associated With Albinism.

Cureus
2025

Unveiling the Immune Landscape: Single-Cell Sequencing of Infectious Mononucleosis Patients.

Journal of medical virology
2025

Onset of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis triggered by sudden reactivation of asymptomatic latent Epstein-Barr virus infection.

Hematology, transfusion and cell therapy
2025

Efficacy and safety of ruxolitinib-based regimen in the treatment of paediatric Epstein-Barr virus-associated haemophagocytic lymphohistiocytosis.

British journal of haematology
2025

Hospitalization trends and clinical burden of paediatric haemophagocytic lymphohistiocytosis in a multicentre study in China.

British journal of haematology
2025

A Rare Presentation of Plasmodium vivax Malaria: Pancytopenia due to Hemophagocytic Syndrome in a Pregnant Woman.

Case reports in medicine
2025

Trimethoprim/sulfamethoxazole and risk of haemophagocytic lymphohistiocytosis (HLH): a literature review and disproportionality analysis using individual case safety reports from FAERS.

The Journal of antimicrobial chemotherapy
2025

Development of an early mortality risk prediction model for pediatric patients with secondary hemophagocytic lymphohistiocytosis.

Italian journal of pediatrics
2025

Lymphoma-Associated Hemophagocytic Syndrome (LAHS) Masquerading as Severe Sepsis and Septic Shock.

Cureus
2025

[Management of side effects of CAR T cells].

Innere Medizin (Heidelberg, Germany)
2025

Tuberculous Meningitis and Hemophagocytic Lymphohistiocytosis in a Patient on Adalimumab: Diagnostic Challenges in the Setting of Suspected Non-Tuberculous Mycobacteria Co-Infection.

European journal of case reports in internal medicine
2025

X-linked lymphoproliferative syndrome type 1 presenting as DRESS-HLH overlap syndrome.

BMJ case reports
2025

A case of acute myeloid leukemia with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis successfully treated by cord blood transplantation.

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

Complications of Chimeric Antigen Receptor T-cell Therapy.

Emergency medicine clinics of North America
2025

Severe drug eruption induced by teicoplanin, complicated by hemophagocytic syndrome: A case report and brief literature review.

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

X-linked lymphoproliferative disease type 1: a clinical and genetic update.

Frontiers in immunology
2025

Emapalumab treatment in rheumatologic disease-associated hemophagocytic lymphohistiocytosis: a plain language summary.

Immunotherapy
2025

A Fog-Delayed Flight and the Story of Sir Michael Anthony Epstein (1921-2024) and Yvonne Margaret Barr (1932-2016).

Archivos argentinos de pediatria
2025

Hemophagocytic Lymphohistiocytosis Secondary to Disseminated Histoplasmosis.

Cureus
2025

Life-Threatening Macrophage Activation Syndrome in Pregnancy: First Manifestation of SLE Induced by Parvovirus B19.

International journal of molecular sciences
2025

A Case of High-Risk Myelodysplastic Syndrome With Cryoglobulinemia, Hemophagocytic Lymphohistiocytosis, and Progression to Multiple Organ Failure.

Journal of medical cases
2025

Body mass index and hemophagocytic lymphohistiocytosis: a risk assessment in HIV-positive leukemia patients.

Annals of medicine and surgery (2012)
2025

A Rare Fatal Case of Hemophagocytic Lymphohistiocytosis With Central Pontine Myelinolysis in the Setting of Epstein-Barr Virus Reactivation.

Cureus
2025

Clinical characteristics, risk factors and predictors of Parvovirus B19 infection in patients with autoimmune rheumatic inflammatory diseases: A nested case-control study.

Clinical immunology (Orlando, Fla.)
2025

Haemophagocytic lymphohistiocytosis (HLH) secondary to measles in an adult with a loss of post-vaccination humoral immunity following rituximab.

The Lancet. Infectious diseases
2025

Secondary haemophagocytic lymphohistiocytosis associated with acute viral hepatitis A - A case series.

Tropical doctor
2025

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

Infection
2025

Fatal HLH in patients with X-linked lymphoproliferative disease 1 due to a novel variant in SH2D1A: case report.

Frontiers in immunology
2026

Expert Perspective: Diagnosis and Treatment of Castleman Disease.

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

Bilateral sino-nasal-orbital fungal necrosis associated with hemophagocytic lymphohistiocytosis.

Orbit (Amsterdam, Netherlands)
2025

Ocrelizumab-Induced Hemophagocytic Lymphohistiocytosis: A Case Report.

Cureus
2025

The pathophysiology of hemophagocytic lympho-histiocytosis (HLH) syndrome and insights from animal models.

Clinical and experimental immunology
2025

Clinical features, course, and risk factors of infection-associated secondary hemophagocytic lymphohistiocytosis.

Infection
2025

Double Trouble: Hemophagocytic Lymphohistiocytosis in a Recent COVID-19 Infection Associated With Epstein-Barr Virus (EBV) Reactivation.

Cureus
2025

Whole-genome sequencing reveals genetic backgrounds in Japanese patients with hemophagocytic lymphohistiocytosis treated with the HLH-2004 protocol.

International journal of hematology
2025

Hematopoietic cell transplantation in pediatric EB virus-associated hemophagocytic lymphohistiocytosis.

International journal of hematology
2025

Severe Dengue Associated With Hemophagocytic Lymphohistiocytosis (HLH) in an Adult ICU.

Cureus
2025

Emerging visceral leishmaniasis patients in mid Southern Himalayan region: A public health concern.

Journal of vector borne diseases
2025

Secondary hemophagocytic lymphohistiocytosis in a 5-month-old infant with IBD post-COVID-19: a case report.

Frontiers in pediatrics
2025

Epstein-Barr virus infection plays a crucial role in triggering hemophagocytic lymphohistiocytosis in patients with X-linked inhibitor of apoptosis protein deficiency.

Hematology (Amsterdam, Netherlands)
2025

[Successful intensive treatment for severe fever with thrombocytopenia syndrome complicated by virus-associated hemophagocytic syndrome in a very elderly patient].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2025

Late presentation of X-linked inhibitor of apoptosis (XIAP) deficiency in a young adult.

Allergy, asthma, and clinical immunology : official journal of the Canadian Society of Allergy and Clinical Immunology
2025

Integration of anti-PD-1 antibody into chemotherapeutic regimens improved the outcome of aggressive NK cell leukemia: a single-center retrospective real-world analysis.

Frontiers in immunology
2025

Systemic EBV+ T-Cell Lymphoma of Childhood with Hemophagocytic Lymphohistiocytosis in a Patient with a Highly Complex Karyotype.

Genes
2025

Human Immune Responses to Epstein-Barr Virus Highlighted by Immunodeficiencies.

Annual review of immunology
2025

Propylthiouracil-induced ANCA-associated vasculitis complicated by granulocytopenia and hemophagocytosis: a case report.

Frontiers in medicine
2025

The lncRNA LINC02446 promotes tumor progression and HLH occurrence by regulating the expression of KLRs and IL-10 in EBV-NK-LPDs.

International immunopharmacology
2025

Epstein-Barr virus infection and prognosis in haemophagocytic lymphohistiocytosis patients with underlying rheumatic diseases: a single-centre retrospective study.

Clinical and experimental rheumatology
2025

[A case of hepatitis-associated aplastic anemia complicated by hemophagocytic lymphohistiocytosis and literature review].

Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics
2025

Management Challenge of Coexistence of Macrophage Activation Syndrome, Systemic Lupus Erythematosus, and Hepatitis B: Case Report.

Clinical case reports
2025

Salvage HLA-haploidentical Peripheral Blood Stem Cell Transplantation Using Post-transplant Cyclophosphamide for Recurrent Hemophagocytic Lymphohistiocytosis-associated Graft Failure after Cord Blood Transplantations.

Internal medicine (Tokyo, Japan)
2025

Study on ferritin and liver injury in pediatric hemophagocytic lymphohistiocytosis associated with infection.

Scientific reports
2025

Less frequent complications following CAR T-cell therapies: hemophagocytic lymphohistiocytosis, graft-versus-host disease, thrombotic microangiopathy, coagulation disorders and secondary malignancies: best practice recommendations from the EBMT Practice Harmonization and Guidelines Committee.

Bone marrow transplantation
2025

Unprecedented case of rapid-onset autoimmune hepatitis triggered by COVID-19 mRNA vaccination, complicated by concurrent severe hemophagocytic lymphohistiocytosis and acute liver failure in a previously healthy adult: a multidisciplinary approach and review of therapeutic interventions.

Clinical journal of gastroenterology
2025

Cytokine Storms in COVID-19, Hemophagocytic Lymphohistiocytosis, and CAR-T Therapy.

JAMA network open
2025

Severe pneumonia caused by Chlamydia abortus complicated by hemophagocytic syndrome: a case report.

Frontiers in medicine
2025

Successful treatment of hemophagocytic lymphohistiocytosis in a trauma patient: a case report.

Journal of trauma and injury
2025

Case Report: A novel IKBKB variant (c.1705G>T) is associated with immune dysregulation and disseminated tuberculosis.

Frontiers in immunology
2025

Chronic Hepatitis and Hemophagocytic Lymphohistiocytosis Associated With Systemic Chronic Active Epstein-Barr Virus Disease.

ACG case reports journal
2025

Hemophagocytic Lymphohistiocytosis with Predominant T-Lymphocytes in Young Child: An Unusual Presentation of Evolving Acute Myeloid Leukemia.

Journal of clinical medicine
2025

Clinicopathological Prognostic Model for Survival in Adult Patients With Secondary Hemophagocytic Lymphohistiocytosis.

European journal of haematology
2025

Hemophagocytic Lymphohistiocytosis Secondary to Dengue Fever.

European journal of case reports in internal medicine
2025

Development and validation of a predictive model for diagnosing EBER-positive lymphoma-associated hemophagocytic lymphohistiocytosis.

BMC cancer
2024

Peripartum COVID-19 & hemophagocytic lymphohistiocytosis: a case report.

Case reports in perinatal medicine
2025

High Epstein-Barr Virus DNA Load in T Cells Predicts Hemophagocytic Lymphohistiocytosis.

The Journal of infectious diseases
2025

Histoplasmosis - More common than we realize.

IDCases
2025

Severe dengue, aneurysmal sub-arachnoid hemorrhage, and hemophagocytic lymphohistiocytosis: a rare case combination.

Einstein (Sao Paulo, Brazil)
2025

Secondary Hemophagocytic Lymphohistiocytosis in severe COVID-19 - a retrospective cohort study.

Scientific reports
2025

Concurrent hemophagocytic lymphohistiocytosis and thrombotic microangiopathy in a patient with Epstein-Barr virus infection and gastric adenocarcinoma: the puzzle started with microangiopathic hemolytic anemia.

Blood coagulation &amp; fibrinolysis : an international journal in haemostasis and thrombosis
2025

Clinical characteristics and prognostic factors in patients with fever of unknown origin who developed secondary haemophagocytic lymphohistiocytosis.

Annals of hematology
2025

Significance of whole-blood EBV DNA status in T/NK-cell lymphoma-associated hemophagocytic lymphohistiocytosis: a single-center retrospective analysis.

Therapeutic advances in hematology
2024

Haemophagocytic lymphohistiocytosis (HLH) secondary to diffuse large B-cell lymphoma: uncommon complication of a common disease.

BMJ case reports
2025

An Unusual Case of Extracavitary/Solid Variant Primary Effusion Lymphoma With Associated Hemophagocytic Lymphohistiocytosis.

Journal of medical cases
2025

Early identification of macrophage activation syndrome in adult-onset Still's disease: a case report and literature review.

Frontiers in medicine
2025

Human inborn errors of immunity underlying Talaromyces marneffei infections: a multicenter, retrospective cohort study.

Frontiers in immunology
2025

Proteomic Analyses in COVID-19-Associated Secondary Hemophagocytic Lymphohistiocytosis.

Critical care explorations
2025

The ability of CD4dimCD8+ T cells to distinguish between Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis and pediatric infectious mononucleosis.

Immunologic research
2024

Cardiovascular complications in chronic active Epstein-Barr virus disease: a case report and literature review.

Frontiers in pediatrics
2024

Peripheral Axonal Neuropathy in Hemophagocytic Lymphohistiocytosis Secondary to Rickettsia conorii Infection.

Cureus

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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. Unraveling the complexities: A comprehensive review of severe fever with thrombocytopenia syndrome and its associated complications.
    iScience· 2026· PMID 41858618mais citado
  2. Epstein-Barr virus infection in children with liver transplantation.
    World journal of hepatology· 2026· PMID 41809485mais citado
  3. The features and prognosis analysis of central nervous system Epstein-Barr virus infection in children with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis.
    European journal of pediatrics· 2026· PMID 41652264mais citado
  4. Prognostic Value of Laboratory Biomarkers in Risk Stratification for Short-Term Outcomes in Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis.
    Current medical science· 2026· PMID 41591714mais citado
  5. Construction of a forest plot prediction model based on Lasso regression for Epstein-Barr virus associated hemophagocytic lymphohistiocytosis in children.
    Italian journal of pediatrics· 2026· PMID 41492100mais citado
  6. A Rare Case of Secondary/Acquired Hemophagocytic Lymphohistiocytosis due to Epstein-Barr Virus Presenting with Neuropsychiatric Features.
    J Assoc Physicians India· 2026· PMID 41818116recente
  7. Successful Management of Suspected Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis in BRAF-Mutant Cholangiocarcinoma Following Treatment With Immune Checkpoint Inhibitors and BRAF/MEK Inhibitors: A Case Report.
    Cancer Rep (Hoboken)· 2026· PMID 41761754recente
  8. The Paucity of Typical Pathology: An Autopsy Series of Typhus Group Rickettsiosis-Associated Hemophagocytic Lymphohistiocytosis.
    Pathogens· 2026· PMID 41754482recente
  9. A 70-Year-Old Woman Receiving Immunosuppressive Therapy Presenting With Iliopsoas Abscess, Pulmonary Miliary Tuberculosis, and Secondary Hemophagocytic Lymphohistiocytosis.
    Am J Case Rep· 2026· PMID 41736384recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:158048(Orphanet)
  2. Busca completa no PubMed(PubMed)
  3. Q55785537(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 hemofagocítico associado a infecção
Compêndio · Raras BR

Síndrome hemofagocítico associado a infecção

ORPHA:158048
Prevalência
Unknown
CID-10
D76.2 · Síndrome hematofagocítica associada à infecção
CID-11
Início
All ages
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0019068
EuropePMC
Wikidata
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