Raras
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Linfo-histiocitose hemofagocítica secundária
ORPHA:158041CID-11 · 3B62.YDOENÇA RARA

Linfo-histiocitose hemofagocítica (uma doença inflamatória séria em que o próprio sistema de defesa do corpo ataca as células do sangue e outros órgãos saudáveis), que pode ser causada por infecções, doenças autoimunes ou cânceres. Os sinais e sintomas incluem febre, inchaço dos gânglios (ínguas), aumento do fígado, aumento do baço e pancitopenia (diminuição de todos os tipos de células do sangue, como glóbulos vermelhos, brancos e plaquetas).

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Introdução

O que você precisa saber de cara

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Linfo-histiocitose hemofagocítica (uma doença inflamatória séria em que o próprio sistema de defesa do corpo ataca as células do sangue e outros órgãos saudáveis), que pode ser causada por infecções, doenças autoimunes ou cânceres. Os sinais e sintomas incluem febre, inchaço dos gânglios (ínguas), aumento do fígado, aumento do baço e pancitopenia (diminuição de todos os tipos de células do sangue, como glóbulos vermelhos, brancos e plaquetas).

Pesquisas ativas
3 ensaios
12 total registrados no ClinicalTrials.gov
Publicações científicas
587 artigos
Último publicado: 2026 Mar
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SUS: Sem cobertura SUSScore: 0%
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Sinais e sintomas

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

Partes do corpo afetadas

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

+ 28 sintomas em outras categorias

Características mais comuns

Autoimunidade
Concentração elevada de alanina aminotransferase circulante
Concentração elevada de aspartato aminotransferase circulante
Hiperbilirrubinemia
Aumento da concentração circulante de interleucina 6
Taxa de sedimentação de eritrócitos elevada
56sintomas
Sem dados (56)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 56 características clínicas mais associadas, ordenadas por frequência.

AutoimunidadeAutoimmunity
Concentração elevada de alanina aminotransferase circulanteElevated circulating alanine aminotransferase concentration
Concentração elevada de aspartato aminotransferase circulanteElevated circulating aspartate aminotransferase concentration
HiperbilirrubinemiaHyperbilirubinemia
Aumento da concentração circulante de interleucina 6Increased circulating interleukin 6 concentration

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico587PubMed
Últimos 10 anos200publicações
Pico202563 papers
Linha do tempo
2026Hoje · 2026🧪 2010Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

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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.
Aprovado1
3Fase 33
2Fase 22
·Pré-clínico4
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 10 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

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Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

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

Outros ensaios clínicos

12 ensaios clínicos encontrados, 3 ativos.

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

Timeline de publicações
525 papers (10 anos)
#1

Neurological Involvement in Adult-Onset Secondary Hemophagocytic Lymphohistiocytosis: Clinical Features and Prognostic Implications.

Brain and behavior2026 Feb

Secondary hemophagocytic lymphohistiocytosis (sHLH) with central nervous system (CNS) involvement poses significant diagnostic and therapeutic challenges. This study aimed to characterize the clinical features, laboratory profiles, and prognostic impact of neurological involvement in adult-onset sHLH. We analyzed 130 adult sHLH patients, comparing 28 with CNS involvement to 102 without neurological manifestations. Clinical parameters, neuroimaging, cerebrospinal fluid (CSF) profiles, cytokine levels, treatment responses, and survival outcomes were evaluated. Patients with CNS involvement were older (median age, 54 vs. 46 years; p = 0.013) and had higher disease severity (median HScore, 250 vs. 210; p < 0.001). Malignancy-associated sHLH was more prevalent in the CNS-positive group (42.9% vs. 29.4%; p = 0.038). Neurological manifestations included altered mental status, impaired consciousness, and seizures. Neuroimaging revealed abnormalities in 71.4% of the cases, primarily T2-weighted fluid-attenuated inversion recovery hyperintensities and leptomeningeal enhancement. CNS-positive patients exhibited markedly elevated inflammatory markers, most notably CSF Interleukin-6 (p < 0.001). In multivariable analysis adjusted for malignancy, age, ferritin, and HScore, CNS involvement independently predicted mortality (adjusted HR = 2.0, 95% CI: 1.1-3.7, p = 0.023), with a significantly shorter median overall survival (6.5 vs. 11.5 months, p < 0.0001). Malignancy-associated etiology and HScore ≥ 250 were also independent prognostic factors. The DEP (dexamethasone, etoposide, and polyethylene glycol-asparaginase) regimen achieved a faster median time to initial response than the HLH-94 protocol (9 vs. 14 days, p = 0.02). CNS involvement defines a severe phenotype of adult-onset sHLH, characterized by malignancy-prone etiology, intense neuroinflammation, and poor prognosis. We establish CNS involvement as an independent predictor of mortality, underscoring the critical need for early recognition and CNS-directed therapies.

#2

Fatal EBV-Associated Posttransplant Lymphoproliferative Disease and Secondary Hemophagocytic Lymphohistiocytosis in a Pediatric Patient With Hurler Syndrome Following Hematopoietic Cell Transplantation: A Case Report and Review of the Literature.

Pediatric blood &amp; cancer2026 Jan
#3

A 70-Year-Old Woman Receiving Immunosuppressive Therapy Presenting With Iliopsoas Abscess, Pulmonary Miliary Tuberculosis, and Secondary Hemophagocytic Lymphohistiocytosis.

The American journal of case reports2026 Feb 25

BACKGROUND Miliary tuberculosis (TB) carries an increased risk in immunosuppressed patients. Extrapulmonary manifestations include hemophagocytic lymphohistiocytosis (HLH) and iliopsoas abscess. The coexistence of multiple extrapulmonary involvements can result in a significant diagnostic challenge. In the treatment of TB-associated HLH, the use of immunosuppressive agents remains controversial because of the risk of exacerbating TB. This report describes the case of a 70-year-old woman with rheumatoid arthritis presenting with an iliopsoas abscess, pulmonary miliary tuberculosis, and secondary HLH. CASE REPORT A 70-year-old Japanese woman with a history of rheumatoid arthritis treated with immunosuppressive therapy including golimumab and iguratimod presented with fever, dyspnea, cough, and weight loss. Imaging demonstrated an iliacus muscle abscess and possible pulmonary miliary tuberculosis. Clinical manifestations, laboratory findings, and bone marrow biopsy results were consistent with a diagnosis of HLH. Acid-fast bacilli cultures were positive for Mycobacterium tuberculosis from the abscess, bronchoalveolar lavage fluid, urine, and blood, confirming the diagnosis of miliary TB. The patient received the HLH-94 treatment protocol and antituberculosis therapy for TB-associated HLH, but unfortunately died of multi-organ failure. CONCLUSIONS In immunosuppressed patients presenting with multi-organ involvement, such as iliopsoas abscess and HLH, clinicians should maintain a high index of suspicion for miliary TB and pursue proactive diagnostic evaluation. Given the limited evidence supporting immunomodulatory therapies in TB-associated HLH, management should be individualized and guided by multidisciplinary discussion.

#4

Case Reports of Visceral Leishmaniasis-Associated Hemophagocytic Lymphohistiocytosis in Adults: A Complex Immune Phenomenon.

Reports (MDPI)2026 Jan 20

Background: Visceral Leishmaniasis (VL), a severe systemic parasitic disease caused by Leishmania species, can be complicated by secondary Hemophagocytic Lymphohistiocytosis (HLH). HLH is a life-threatening hyperinflammatory syndrome characterized by excessive immune activation that results in multiorgan dysfunction. The co-occurrence of VL and HLH in adults is a rare but critical diagnostic and therapeutic challenge, often leading to fatal outcomes if treatment is delayed. Case Presentation: We present two cases of adult males (60 and 72 years old) from Greece, an endemic area for L. infantum, who presented with prolonged fever, pancytopenia, hepatosplenomegaly, and impaired liver function. Both patients exhibited extremely elevated ferritin (all > 2000 ng/mL and one > 20,000 ng/mL) and hypertriglyceridemia, fulfilling key laboratory criteria for HLH. Diagnosis was confirmed by the visualization of Leishmania amastigotes in bone marrow aspirates, which also demonstrated features of hemophagocytosis. Case 1, critically ill with acute kidney injury and coagulopathy, required combined treatment with liposomal Amphotericin B and immunoglobulin therapy for HLH. Case 2, who showed rapid and "spectacular improvement" solely after receiving liposomal Amphotericin B, did not require HLH-specific immunosuppression. Conclusions: VL-associated HLH should be considered in adult patients presenting with complex systemic inflammation, fever, and cytopenias, particularly in endemic settings. Our cases illustrate that the prompt initiation of anti-leishmanial therapy with liposomal Amphotericin B can be sufficient to reverse the HLH syndrome by eliminating the infectious trigger. However, intensive immunomodulation may be necessary in patients presenting with critical multi-organ failure.

#5

Avoidance of related donors in CAEBV with germline immune variants: long-termoutcome of matched unrelated donor HSCT - a case report.

Annals of hematology2026 Feb 19

This study reports an adult-onset case of NK cell-predominant chronic active Epstein-Barr virus infection (CAEBV) harboring multiple heterozygous germline variants affecting antiviral immunity. Functional assessments of NK cell cytotoxicity and degranulation in the patient and her family members revealed subclinical immune defects in several relatives, leading to the exclusion of related donors. The patient ultimately underwent a fully HLA-matched unrelated donor hematopoietic stem cell transplantation (MUD-HSCT), achieving early virologic remission and complete donor chimerism. However, the post-transplant course was complicated by severe immune-related adverse events, including acute and chronic graft-versus-host disease (GVHD), thrombotic microangiopathy, viral reactivations, and secondary hemophagocytic lymphohistiocytosis, ultimately resulting in death due to severe pulmonary infection and multi-organ failure. This case underscores the critical role of immunogenetic risk stratification in guiding transplant decisions. Matched unrelated donor transplantation, supported by comprehensive functional and genetic screening, offers curative potential while avoiding the use of immunologically compromised donors. Nevertheless, long-term outcomes in CAEBV depend not only on virologic remission but also on sustained immune reconstitution. In addition, this report reviews precision transplantation strategies that integrate host genetic background, immune function, and viral dynamics, providing a roadmap for the future management of CAEBV.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC348 artigos no totalmostrando 199

2026

Chronic granulomatous disease with suspected secondary hemophagocytic lymphohistiocytosis: a case report and hypothesis-generating rationale for neuroimmune modulation.

Critical care (London, England)
2026

A 70-Year-Old Woman Receiving Immunosuppressive Therapy Presenting With Iliopsoas Abscess, Pulmonary Miliary Tuberculosis, and Secondary Hemophagocytic Lymphohistiocytosis.

The American journal of case reports
2026

Case Reports of Visceral Leishmaniasis-Associated Hemophagocytic Lymphohistiocytosis in Adults: A Complex Immune Phenomenon.

Reports (MDPI)
2026

Avoidance of related donors in CAEBV with germline immune variants: long-termoutcome of matched unrelated donor HSCT - a case report.

Annals of hematology
2026

Secondary hemophagocytic lymphohistiocytosis following blood transfusion in the absence of an identifiable trigger.

SAGE open medical case reports
2026

Interferon-gamma blocking as a promising treatment for severe liver dysfunction in secondary hemophagocytic lymphohistiocytosis after liver transplantation.

JPGN reports
2026

Hemophagocytic lymphohistiocytosis secondary to isolated bronchial infection caused by Mycobacterium marseillense in a patient with GATA2 missense mutation: A rare case report.

Journal of infection and public health
2026

Cytomegalovirus Infection-Associated Hemophagocytic Lymphohistiocytosis and Histiocytic Necrotising Lymphadenitis Progressing to Systemic Lupus Erythematosus: A Case Report.

Cureus
2026

Navigating a Dual Hyperinflammatory State: Overlap of Drug Reaction With Eosinophilia and Systemic Symptoms (DRESS) and Secondary Hemophagocytic Lymphohistiocytosis (HLH) in a Child.

Cureus
2026

An Unusual Presentation of Secondary Hemophagocytic Lymphohistiocytosis With Melena: A Case Report.

Cureus
2026

Secondary hemophagocytic lymphohistiocytosis concurrent with febrile infection-related epilepsy syndrome in a child.

Seizure
2026

Combination therapy success in refractory pediatric visceral leishmaniasis with hemophagocytic lymphohistiocytosis: A case report.

Medicine
2026

Neurological Involvement in Adult-Onset Secondary Hemophagocytic Lymphohistiocytosis: Clinical Features and Prognostic Implications.

Brain and behavior
2026

Secondary hemophagocytic lymphohistiocytosis in patients treated with peritoneal dialysis.

Polish archives of internal medicine
2025

Influenza B-Triggered Secondary Hemophagocytic Lymphohistiocytosis in an Adult Male: A Diagnostic Challenge.

Cureus
2026

Secondary hemophagocytic lymphohistiocytosis in a patient with severe leptospirosis: a case report.

Journal of medical case reports
2026

A case report of hemophagocytic lymphohistiocytosis occurring during ibrutinib chemotherapy with a literature review.

Medicine
2026

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

Annals of medicine
2025

Presentation, Diagnosis, and Acute Treatment of Secondary Hemophagocytic Lymphohistiocytosis: A Case Report.

Cureus
2025

[Clinical Characteristics and Prognosis of Children with Hematological Malignancies Complicated by Secondary Hemophagocytic Lymphohistiocytosis].

Zhongguo shi yan xue ye xue za zhi
2025

Retrospective Multicenter Study of Human Granulocytic Anaplasmosis, France, 2012-2024.

Emerging infectious diseases
2026

Corrigendum to Analysis of risk factors for the early death of lymphocyte subsets in adult patients with secondary hemophagocytic lymphohistiocytosis Experimental Hematology Volume 153 (2025), 105286.

Experimental hematology
2025

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

Reports (MDPI)
2025

Intravascular Large B-cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis and Pyrexia of Unknown Origin: A Diagnostic Dilemma.

Cureus
2025

Adult-Onset Still's Disease Complicated by Secondary Hemophagocytic Lymphohistiocytosis: A Case Report.

Clinical case reports
2026

Efficacy of Etoposide-Based Therapy in Pediatric Secondary Hemophagocytic Lymphohistiocytosis.

European journal of haematology
2026

Improving Recognition of Secondary Hemophagocytic Lymphohistiocytosis (HLH) through a Ferritin-Based Automated Alert: An Interrupted Time Series Analysis.

Clinical chemistry
2025

Secondary Hemophagocytic Lymphohistiocytosis in a Patient With Crohn's Disease Receiving Infliximab: A Diagnostic Challenge.

Cureus
2025

Therapeutic Plasma Exchange in Pediatric Care: Insights from a Tertiary Care Centre Experience.

Indian pediatrics
2025

Perforin Expression and Natural Killer-Cell Proportion as Biomarkers in Secondary Hemophagocytic Lymphohistiocytosis.

Medical science monitor : international medical journal of experimental and clinical research
2025

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

ASM case reports
2025

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

Journal of medical cases
2026

Fatal EBV-Associated Posttransplant Lymphoproliferative Disease and Secondary Hemophagocytic Lymphohistiocytosis in a Pediatric Patient With Hurler Syndrome Following Hematopoietic Cell Transplantation: A Case Report and Review of the Literature.

Pediatric blood &amp; cancer
2025

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

Annals of hematology
2025

Characteristics of Secondary Malignancy Among Children With Primary Immunodeficiency Disorders in Saudi Arabia.

Cureus
2026

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

Experimental hematology
2025

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

Annals of hematology
2025

Case Report: Longitudinal mass cytometry profiling of a patient with disseminated histoplasmosis and secondary hemophagocytic lymphohistiocytosis.

Frontiers in immunology
2026

Pediatric Severe Eosinophilia: Etiological Spectrum, Diagnostic Algorithm, and Case-Based Insights From a Tertiary Care Center.

International journal of laboratory hematology
2025

Cytomegalovirus Viremia as A Cause of Fever of Unknown Origin in an Immunocompetent Adult.

European journal of case reports in internal medicine
2025

Secondary Hemophagocytic Lymphohistiocytosis Triggered by Subdural Empyema due to Streptococcus constellatus: A Rare Triad.

Journal of Brown hospital medicine
2025

Massive suprachoroidal hemorrhage in dengue-associated HLH: A clinical image.

IDCases
2025

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
2025

Diagnosis and treatment of secondary hemophagocytic lymphohistiocytosis in adults.

Clinical advances in hematology &amp; oncology : H&amp;O
2025

Laboratory diagnosis of 44 cases of pediatric histiocytic necrotizing lymphadenitis.

Frontiers in pediatrics
2025

Prelude to the perfect storm: the many triggers of secondary hemophagocytic lymphohistiocytosis.

Postgraduate medicine
2025

Imaging in Hemophagocytic Lymphohistiocytosis: A Comprehensive Pictorial Essay.

Cureus
2025

Sweet Syndrome-like Dermatosis as a Precursor to Overlapping Hematologic Malignancies: A Case Report and Review.

Journal of clinical medicine
2025

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

Pediatric transplantation
2025

Hodgkin Lymphoma: An Unusual Presentation With Hemophagocytic Lymphohistiocytosis.

Cureus
2025

Prognostic value of blood inflammatory composite markers in the survival of pediatric patients with secondary hemophagocytic lymphohistiocytosis.

Frontiers in pediatrics
2025

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

Case reports in infectious diseases
2025

A Comprehensive Guide to Typhoidal Anemia.

Prague medical report
2025

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

Italian journal of pediatrics
2025

Secondary hemophagocytic lymphohistiocytosis associated with adjuvant pembrolizumab therapy in a young patient with triple-negative breast cancer: a case report with literature review.

Archives of gynecology and obstetrics
2025

Characteristics of 21 Patients with Secondary Hemophagocytic Lymphohistiocytosis-Insights from a Single-Center Retrospective Study.

Medicina (Kaunas, Lithuania)
2025

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

Annals of medicine and surgery (2012)
2025

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

Infection
2025

Ruxolitinib targets JAK-STAT signaling to modulate neutrophil activation in refractory macrophage activation syndrome.

Blood
2025

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

Frontiers in pediatrics
2025

Perspectives on the current diagnostic and treatment paradigms in secondary hemophagocytic lymphohistiocytosis (HLH).

Orphanet journal of rare diseases
2025

Deep insight into cytokine storm: from pathogenesis to treatment.

Signal transduction and targeted therapy
2025

Recognizing and Managing Secondary Hemophagocytic Lymphohistiocytosis in Adults: A Practical Clinical Guide.

Hematology/oncology clinics of North America
2025

Macrophage Activation Syndrome as an Atypical Manifestation of Mixed Connective Tissue Disease in a 14-Year-Old Girl: A Case Report.

Cureus
2025

Methylmalonic acidemia with recurrent hemophagocytic lymphohistiocytosis: a case report and review of the literature.

BMC pediatrics
2025

[Secondary hemophagocytic lymphohistiocytosis due to icteric leptospirosis with acute renal failure].

Deutsche medizinische Wochenschrift (1946)
2025

Immune Effector Cell-associated Hemophagocytic Lymphohistiocytosis-like Syndrome (IEC-HS).

Hematology/oncology clinics of North America
2025

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

European journal of haematology
2024

Disseminated Histoplasmosis with Secondary Hemophagocytic Lymphohistiocytosis in a Renal Transplant Recipient on Belatacept-based Immunosuppression.

Journal of Brown hospital medicine
2025

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

Einstein (Sao Paulo, Brazil)
2025

Babesiosis with low parasitemia as a cause of secondary hemophagocytic lymphohistiocytosis in a previously healthy adult.

IDCases
2025

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

Scientific reports
2025

Tetrandrine alleviates macrophage activation syndrome after CAR-T cell therapy.

Phytomedicine : international journal of phytotherapy and phytopharmacology
2025

Aggressive T-cell Lymphoma Smoldering As Hemophagocytic Lymphohistiocytosis: A Diagnostic and Medical Challenge.

Cureus
2025

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

Critical care explorations
2025

Intercontinentally validated diagnostic criteria for secondary hemophagocytic lymphohistiocytosis-So welcome!

Journal of internal medicine
2025

Multicenter validation of secondary hemophagocytic lymphohistiocytosis diagnostic criteria.

Journal of internal medicine
2025

Preoperative pembrolizumab‑induced hemophagocytic lymphohistiocytosis in a patient with breast cancer: A case report.

Oncology letters
2024

COX Regression Analysis and Mortality Risk Prediction Model of 85 Adult Patients with Secondary Hemophagocytic Lymphohistiocytosis.

British journal of hospital medicine (London, England : 2005)
2025

PRMT5 inhibition reduces hyperinflammation in a murine model of secondary hemophagocytic lymphohistiocytosis.

Blood advances
2025

Macrophage Activation Syndrome/Secondary Hemophagocytic Lymphohistiocytosis in Adult-Onset Still's Disease: An Uncommon Initial Presentation in a Young Nepalese Female: A Case Report.

Clinical case reports
2025

Secondary Hemophagocytic Lymphohistiocytosis Syndrome Developing in a Patient With Chronic Lymphocytic Leukemia Under a Long-term Ibrutinib Therapy: A Case Report and Literature Review.

Journal of immunotherapy (Hagerstown, Md. : 1997)
2024

Hemophagocytic Lymphohistiocytosis Secondary to Miliary Tuberculosis in a Resource-Limited Setting: A Case Report.

Cureus
2024

Case report: A cyclic neutropenia patient with ELANE mutation accompanied by hemophagocytic lymphohistiocytosis.

Frontiers in immunology
2024

A rare case of complicated typhoid fever presenting with secondary Hemophagocytic Lymphohistiocytosis.

Oxford medical case reports
2024

Two-Month Duration of Esophageal Button Battery Impaction in a 23-Month-Old Boy: A Case Report.

Cureus
2024

A vector borne, airborne and food borne infection with secondary hemophagocytic lymphohistocytosis: Case of triple infections in an immuno-competent patient.

Indian journal of medical microbiology
2024

Hemophagocytic Lymphohistiocytosis (HLH): A Rare Complication of Dengue Hemorrhagic Fever.

Cureus
2024

Treatment of Secondary Hemophagocytic Lymphohistiocytosis Associated With Diffuse Large B-cell Lymphoma Using Loncastuximab Tesirine As Lymphoma-Directed Therapy.

Cureus
2025

A Rare Case of Disseminated Strongyloidiasis Leading to Multisystem Organ Failure Following Deceased Donor Kidney Transplantation.

The American surgeon
2025

Enteric Fever with Intussusception, Myocardial Dysfunction, Encephalopathy, and Hemophagocytic Lymphohistiocytosis in an Indian Child.

The American journal of tropical medicine and hygiene
2025

A novel mutation in a craniofacial pustular pyoderma gangrenosum patient with secondary hemophagocytic lymphohistiocytosis.

Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG
2024

CircMETTL3-156aa reshapes the glycolytic metabolism of macrophages to promote M1 polarization and induce cytokine storms in sHLH.

Cell death discovery
2024

The HScore to Diagnose HLH in Scrub Typhus: Overdiagnosis or under Diagnosis and Does It Really Matter?

Indian journal of critical care medicine : peer-reviewed, official publication of Indian Society of Critical Care Medicine
2024

Predicting Secondary Hemophagocytic Lymphohistiocytosis in Adult Patients with Scrub Typhus and Its Prognostic Significance.

Indian journal of critical care medicine : peer-reviewed, official publication of Indian Society of Critical Care Medicine
2025

Peripheral blood smear reveals human granulocytic anaplasmosis, a rare cause of hemophagocytic lymphohistiocytosis.

American journal of hematology
2024

Secondary Hemophagocytic Lymphohistiocytosis Following Dostarlimab Treatment in a Patient With Metastatic Endometrial Cancer.

World journal of oncology
2025

Immune biomarkers and secondary hemophagocytic lymphohistiocytosis: a scoping review protocol.

JBI evidence synthesis
2025

EBV-triggered secondary macrophage activation syndrome in the backdrop of lupus in an adult.

Indian journal of pathology &amp; microbiology
2024

[Effect of Plasma Epstein-Barr Virus Nucleic Acid Loads on the Clinical Features and Prognosis in Adult Secondary Hemophagocytic Lymphohistiocytosis].

Zhongguo shi yan xue ye xue za zhi
2024

Identification of biomarkers for COVID-19 associated secondary hemophagocytic lymphohistiocytosis.

bioRxiv : the preprint server for biology
2024

Hemophagocytic Lymphohistiocytosis Unmasking Systemic Lupus Erythematosus: Management With Belimumab and a Case Study.

Cureus
2024

Secondary hemophagocytic lymphohistiocytosis triggered by Staphylococcus aureus bacteremia: A case report and systemic review.

IDCases
2024

Other Immunomodulatory Treatment for Cytokine Storm Syndromes.

Advances in experimental medicine and biology
2024

IL-1 Family Blockade in Cytokine Storm Syndromes.

Advances in experimental medicine and biology
2024

Etoposide Therapy of Cytokine Storm Syndromes.

Advances in experimental medicine and biology
2024

Cytokine Storm and Sepsis-Induced Multiple Organ Dysfunction Syndrome.

Advances in experimental medicine and biology
2024

Genetics of Acquired Cytokine Storm Syndromes : Secondary HLH Genetics.

Advances in experimental medicine and biology
2024

Human monocytotropic ehrlichiosis-A systematic review and analysis of the literature.

PLoS neglected tropical diseases
2024

Disseminated tuberculosis complicated by intramuscular abscesses, meningoencephalitis, and hemophagocytic lymphohistiocytosis: a case report.

BMC infectious diseases
2024

Unleashing the Cytokine Storm: A Case of Macrophage Activation Syndrome in Systemic Lupus Erythematosus.

Cureus
2024

Macrophage Activation Syndrome in Viral Sepsis.

Viruses
2024

CRP and sCD25 help distinguish between adult-onset Still's disease and HLH.

European journal of haematology
2024

Unmasking the Culprits: A Case of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis Presenting With Mouth Ulcers and Nosebleeds.

Cureus
2024

Comprehensive mapping of immune perturbations associated with secondary hemophagocytic lymphohistiocytosis.

Journal of leukocyte biology
2024

From multisystem inflammatory syndrome in children to secondary hemophagocytic lymphohistiocytosis a series of misfortunate events: case report and review of literature.

Sudanese journal of paediatrics
2024

Diarrhea as the Initial Presentation in a Patient With HIV Diagnosed With Hodgkin's Lymphoma.

Cureus
2024

A Retrospective Review of Secondary Hemophagocytic Lymphohistiocytosis (HLH) and Dengue-associated HLH from a Teaching Hospital in Singapore.

Clinical hematology international
2024

Single center clinical analysis of macrophage activation syndrome complicating juvenile rheumatic diseases.

Pediatric rheumatology online journal
2024

Management of hematological patients requiring emergency chemotherapy in the intensive care unit.

Intensive care medicine
2024

Malignancy-Associated Secondary Hemophagocytic Lymphohistiocytosis Mimicking an Infection: A Case Report and Review of the Literature.

Cureus
2024

A Case of Secondary Hemophagocytic Lymphohistiocytosis in a Patient With T-cell Lymphoma.

Cureus
2024

Comprehensive evaluation of immune dysregulation in secondary hemophagocytic lymphohistiocytosis.

Virulence
2024

Marginal Zone Lymphoma Manifesting as Macrophage Activation Syndrome: A Case Report.

Journal of investigative medicine high impact case reports
2024

Low interleukin-10 level indicates a good prognosis in Salmonella enterica serovar typhimurium-induced pediatric hemophagocytic lymphohistiocytosis: A case report.

World journal of clinical cases
2024

A 14-step desensitization protocol for sebelipase alfa hypersensitivity in a patient with Wolman disease and secondary hemophagocytic lymphohistiocytosis.

Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology
2024

Tuberculosis-Associated Hemophagocytic Lymphohistiocytosis: Diagnostic Challenges and Determinants of Outcome.

Open forum infectious diseases
2024

Probable secondary hemophagocytic lymphohistiocytosis manifesting as central nervous system lesions after COVID-19 vaccination: a case report.

Frontiers in neurology
2024

Ruxolitinib, IV Immunoglobulin, and High-Dose Glucocorticoids for Critically Ill Adults With Secondary Hemophagocytic Lymphohistiocytosis: A Single-Center Observational Pilot Study.

Critical care explorations
2024

The Systemic Score May Identify Life-Threatening Evolution in Still Disease: Data from the GIRRCS AOSD-Study Group and the AIDA Network Still Disease Registry.

Arthritis &amp; rheumatology (Hoboken, N.J.)
2024

Hemophagocytic Lymphohistiocytosis Associated With Human Herpesvirus-6 (HHV-6) Infection in an Immunocompetent Adult: A Case Report.

Cureus
2024

Secondary hemophagocytic lymphohistiocytosis in pediatric patients with visceral leishmaniasis and Epstein-Barr virus infection.

Annals of hematology
2024

Dengue Hemorrhagic Fever Causing Postpartum Hemorrhage and Hemophagocytic Lymphohistiocytosis in a Young Woman: A Case Report.

Cureus
2024

[Combined immunosuppression with cyclosporin A, mycophenolate mofetil (MMF) and dexamethasone for activity control of recurrent secondary hemophagocytic lymphohistiocytosis (sHLH) with underlying systemic lupus erythematosus (SLE)].

Innere Medizin (Heidelberg, Germany)
2024

Severe Lymphoma-Associated Hemophagocytic Syndrome in a Young Woman.

Cureus
2024

Immunopathology of and potential therapeutics for secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome: a translational perspective.

Experimental &amp; molecular medicine
2024

Association of a decreased platelet count with poor survival in patients with adult secondary hemophagocytic lymphohistiocytosis.

Annals of hematology
2024

Value of hemophagocytosis in the diagnosis of hemophagocytic lymphohistiocytosis in critically ill patients.

European journal of haematology
2023

Hemophagocytic lymphohistiocytosis: A scourge for the physician and bane to the bone marrow.

Annals of African medicine
2024

Comparison of different treatment regimens and analysis of prognostic factors in secondary hemophagocytic lymphohistiocytosis in adults: A single-center retrospective study.

Advances in clinical and experimental medicine : official organ Wroclaw Medical University
2023

Case Report: Successful avoidance of etoposide for primary hemophagocytic lymphohistiocytosis-induced multiple organ dysfunction syndrome using emapalumab.

Frontiers in pediatrics
2024

Case Report: A Case of Complicated Typhoid Fever with Rhabdomyolysis and Hemophagocytic Lymphohistiocytosis.

The American journal of tropical medicine and hygiene
2024

Performances of the H-score and the HLH-2004 score in the positive diagnosis of secondary hemophagocytic lymphohistiocytosis.

Current research in translational medicine
2023

A 10-Year Retrospective Study on Pediatric Visceral Leishmaniasis in a European Endemic Area: Diagnostic and Short-Course Therapeutic Strategies.

Healthcare (Basel, Switzerland)
2023

Q fever, a rare cause of secondary hemophagocytic lymphohistiocytosis.

GMS infectious diseases
2023

Elevated monocyte HLA-DR in pediatric secondary hemophagocytic lymphohistiocytosis: a retrospective study.

Frontiers in immunology
2023

[Analysis of High-Risk Clinical Factors of Early Death in Secondary Hemophagocytic Lymphohistiocytosis].

Zhongguo shi yan xue ye xue za zhi
2024

Foamy Macrophages in a Case of Mononucleosis With Amoxicillin-Induced Rash, Hyperlipidemia, and Hemophagocytic Lymphohistiocytosis.

The American Journal of dermatopathology
2023

Hemophagocytic Lymphohistiocytosis: A Rare Complication of COVID-19 in a Patient With Sickle Cell Anemia.

Cureus
2023

Severe Acute Liver Injury due to Secondary Hemophagocytic Lymphohistiocytosis: A Case Report.

GE Portuguese journal of gastroenterology
2023

Solid organ graft-versus-host disease in a recipient of a COVID-19 positive liver graft.

Journal of liver transplantation
2024

Epithelioid granulomas with proliferating T-lymphocytes in bone marrow in a patient with infectious mononucleosis modified by secondary hemophagocytic lymphohistiocytosis.

Pathology international
2023

The signature and predictive value of immune parameters in patients with secondary hemophagocytic lymphohistiocytosis.

Immunobiology
2023

An elevated HScore is associated with poor clinical outcomes in COVID-19.

Revista medica de Chile
2023

Outcome of patients with primary and secondary hemophagocytic lymphohistiocytosis: A retrospective analysis from a tertiary care center.

Medicine
2023

Secondary Hemophagocytic Lymphohistiocytosis: A Series of Three Cases.

Cureus
2024

Hemophagocytic lymphohistiocytosis-how common and how severe is it as a complication of malaria? Retrospective case series and review of the literature.

Infection
2023

Severe acute herpes virus type 2 primo-infection and its association with anti-type 1 interferon autoantibodies.

European journal of clinical microbiology &amp; infectious diseases : official publication of the European Society of Clinical Microbiology
2024

Genetic and clinical characteristics of primary hemophagocytic lymphohistiocytosis in children.

Annals of hematology
2023

Rare case of secondary hemophagocytic lymphohistiocytosis in a patient with disseminated histoplasmosis.

Pediatric investigation
2024

CLINICAL UTILITY OF INTERLEUKIN-1 INHIBITORS IN PEDIATRIC SEPSIS.

Shock (Augusta, Ga.)
2023

Sepsis as the Grand Mimic of Secondary Hemophagocytic Lymphohistiocytosis: Serratia marcescens Bacteremia with Concomitant Decompensated Cirrhotic Liver Disease.

Case reports in critical care
2023

Ehrlichia-Induced Hemophagocytic Lymphohistiocytosis (HLH) With No Response to Doxycycline Treatment.

Cureus
2023

Secondary hemophagocytic lymphohistiocytosis in children (Review).

Experimental and therapeutic medicine
2023

Secondary Hemophagocytic Lymphohistiocytosis Due to Typhoid Fever.

Cureus
2023

[Clinical features and risk factors for secondary hemophagocytic lymphohistiocytosis in elderly patients with severe SARS-CoV-2 infection: a multicenter retrospective cohort study].

Zhonghua wei zhong bing ji jiu yi xue
2023

Acute Liver Failure Caused by Secondary Hemophagocytic Lymphohistiocytosis After COVID-19 Vaccination - Case Report and Literature Review.

International medical case reports journal
2023

A novel prognosis-prediction model based on coagulation indicators in secondary hemophagocytic lymphohistiocytosis.

Annals of hematology
2023

Secondary hemophagocytic lymphohistiocytosis: an unusual complication in disseminated Mycobacterium tuberculosis.

Clinical medicine (London, England)
2023

CXCL9 inhibition does not ameliorate disease in murine models of both primary and secondary hemophagocytic lymphohistiocytosis.

Scientific reports
2023

Hemophagocytic inflammatory syndrome in ADA-SCID: report of two cases and literature review.

Frontiers in immunology
2023

Anakinra versus etoposide-based therapy added to high-dose steroids for the treatment of secondary hemophagocytic lymphohistiocytosis.

European journal of haematology
2023

[Expression and Clinical Significance of Serum sFas/sFasL in Patients with Secondary Hemophagocytic Lymphohistiocytosis].

Zhongguo shi yan xue ye xue za zhi
2023

A Diagnostic Dilemma: Adult-Onset Still's Disease With Secondary Hemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome?

Journal of medical cases
2023

Clinical value of soluble fms-like tyrosine kinase 1 (sFlt-1) in adult secondary hemophagocytic lymphohistiocytosis.

Journal of the Chinese Medical Association : JCMA
2023

Case report: Sulfasalazine-induced hypersensitivity.

Frontiers in medicine
2023

Risk factors and prognosis of early death in secondary hemophagocytic lymphohistiocytosis.

Annals of hematology
2023

Prognostic value of the albumin-bilirubin score in patients with non-Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis.

Frontiers in immunology
2023

Secondary hemophagocytic lymphohistiocytosis associated with heat stroke: A case report and review of literature.

Medicine
2023

A predictive model for identifying secondary underlying diseases of hemophagocytic lymphohistiocytosis.

Frontiers in immunology
2023

Primary Gastrointestinal Diffuse Large B-cell Lymphoma Presenting as Ileal Perforation.

Cureus
2023

Presentations and outcomes of familial hemophagocytic lymphohistiocytosis in the pediatric intensive care units (PICUs).

Frontiers in pediatrics
2023

Postpartum fevers, a rare presentation of secondary hemophagocytic lymphohistiocytosis.

Clinical case reports
2023

Variations in Macrophage Activation Syndrome-associated Cardiac Diseases: A Report on Two Cases.

Iranian journal of immunology : IJI
2023

Prognostic value of lipid profile in adult hemophagocytic lymphohistiocytosis.

Frontiers in oncology
2023

Hemophagocytic lymphohistiocytosis in gastric cancer: A rare syndrome for the oncologist. Case report and brief review.

Frontiers in oncology
2023

Severe COVID-19-Induced Hemophagocytic Lymphohistiocytosis.

Cureus
2023

Severe COVID-19-associated hyperinflammatory syndrome versus classic hemophagocytic lymphohistiocytosis: similarities, differences, and the way forward.

Journal of investigative medicine : the official publication of the American Federation for Clinical Research
2023

A Case of Disseminated Histoplasmosis From California, in the Setting of Secondary Hemophagocytic Lymphohistiocytosis: A Diagnostic Challenge.

Journal of investigative medicine high impact case reports
2023

Acute liver failure and unique challenges of pediatric liver transplantation amidst a worldwide cluster of adenovirus-associated hepatitis.

American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons
2023

Acanthamoeba meningoencephalitis causing secondary hemophagocytic lymphohistiocytosis in an immunocompetent patient: A first case report.

The journal of the Royal College of Physicians of Edinburgh
2022

Disseminated Cytomegalovirus Infection in a Child with Langerhans Cell Histiocytosis.

Journal of global infectious diseases
2023

Prolonged Thrombocytopenia in a Case of MIS-C in a Vaccinated Child.

Journal of investigative medicine high impact case reports
2022

Secondary hemophagocytic lymphohistiocytosis due to Heartland virus.

BMJ case reports
2023

Management of Dengue: An Updated Review.

Indian journal of pediatrics
2022

Hemophagocytic Lymphohistiocytosis in a Patient With Post-acute COVID-19 Infection.

Cureus
2022

Case report: Drug reaction with eosinophilia and systemic symptoms (DRESS)-induced hemophagocytic disorder.

Frontiers in pharmacology
2022

Secondary hemophagocytic lymphohistiocytosis following Zolgensma therapy: An evolving story on the innate response to systemic gene therapy.

Molecular therapy : the journal of the American Society of Gene Therapy
2022

Secondary hemophagocytic lymphohistiocytosis triggered by peripheral T-cell lymphoma: An unusual case report.

Clinical case reports
Ver todos os 348 no EuropePMC

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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. Neurological Involvement in Adult-Onset Secondary Hemophagocytic Lymphohistiocytosis: Clinical Features and Prognostic Implications.
    Brain and behavior· 2026· PMID 41612779mais citado
  2. Fatal EBV-Associated Posttransplant Lymphoproliferative Disease and Secondary Hemophagocytic Lymphohistiocytosis in a Pediatric Patient With Hurler Syndrome Following Hematopoietic Cell Transplantation: A Case Report and Review of the Literature.
    Pediatric blood &amp; cancer· 2026· PMID 41199500mais citado
  3. A 70-Year-Old Woman Receiving Immunosuppressive Therapy Presenting With Iliopsoas Abscess, Pulmonary Miliary Tuberculosis, and Secondary Hemophagocytic Lymphohistiocytosis.
    The American journal of case reports· 2026· PMID 41736384mais citado
  4. Case Reports of Visceral Leishmaniasis-Associated Hemophagocytic Lymphohistiocytosis in Adults: A Complex Immune Phenomenon.
    Reports (MDPI)· 2026· PMID 41718280mais citado
  5. Avoidance of related donors in CAEBV with germline immune variants: long-termoutcome of matched unrelated donor HSCT - a case report.
    Annals of hematology· 2026· PMID 41708804mais citado
  6. Secondary Hemophagocytic Lymphohistiocytosis in a Rocky Mountain Spotted Fever Patient.
    Cureus· 2026· PMID 41994746recente
  7. Pneumococcal HUS in infancy masking DGKE-related thrombotic microangiopathy with secondary hemophagocytic lymphohistiocytosis.
    Pediatr Nephrol· 2026· PMID 41979714recente
  8. Clinical Profile and Outcome of Children with Scrub typhus: A Tertiary Care Centre Observation.
    J Trop Pediatr· 2026· PMID 41968754recente
  9. Secondary Haemophagocytic Lymphohistiocytosis Triggered by Adult-Onset Still's Disease in Mid-trimester Pregnancy Initially Presenting as an Upper Respiratory Tract Infection: A Case Report.
    Cureus· 2026· PMID 41943781recente
  10. Secondary Hemophagocytic Lymphohistiocytosis as the Initial Presentation of Metastatic Carcinoma of Likely Colorectal Origin.
    J Brown Hosp Med· 2026· PMID 41940219recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:158041(Orphanet)
  2. MONDO:0015542(MONDO)
  3. GARD:20026(GARD (NIH))
  4. Busca completa no PubMed(PubMed)
  5. Q55785536(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

Linfo-histiocitose hemofagocítica secundária
Compêndio · Raras BR

Linfo-histiocitose hemofagocítica secundária

ORPHA:158041 · MONDO:0015542
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UMLS
C0019068
EuropePMC
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