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).
Introdução
O que você precisa saber de cara
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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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 28 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 56 características clínicas mais associadas, ordenadas por frequência.
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
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Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Linfo-histiocitose hemofagocítica secundária
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Publicações mais relevantes
Neurological Involvement in Adult-Onset Secondary Hemophagocytic Lymphohistiocytosis: Clinical Features and Prognostic Implications.
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.
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.
A 70-Year-Old Woman Receiving Immunosuppressive Therapy Presenting With Iliopsoas Abscess, Pulmonary Miliary Tuberculosis, and Secondary Hemophagocytic Lymphohistiocytosis.
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.
Case Reports of Visceral Leishmaniasis-Associated Hemophagocytic Lymphohistiocytosis in Adults: A Complex Immune Phenomenon.
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.
Avoidance of related donors in CAEBV with germline immune variants: long-termoutcome of matched unrelated donor HSCT - a case report.
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
Secondary Hemophagocytic Lymphohistiocytosis in a Rocky Mountain Spotted Fever Patient.
Pneumococcal HUS in infancy masking DGKE-related thrombotic microangiopathy with secondary hemophagocytic lymphohistiocytosis.
Clinical Profile and Outcome of Children with Scrub typhus: A Tertiary Care Centre Observation.
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.
Secondary Hemophagocytic Lymphohistiocytosis as the Initial Presentation of Metastatic Carcinoma of Likely Colorectal Origin.
📚 EuropePMC348 artigos no totalmostrando 199
Chronic granulomatous disease with suspected secondary hemophagocytic lymphohistiocytosis: a case report and hypothesis-generating rationale for neuroimmune modulation.
Critical care (London, England)A 70-Year-Old Woman Receiving Immunosuppressive Therapy Presenting With Iliopsoas Abscess, Pulmonary Miliary Tuberculosis, and Secondary Hemophagocytic Lymphohistiocytosis.
The American journal of case reportsCase Reports of Visceral Leishmaniasis-Associated Hemophagocytic Lymphohistiocytosis in Adults: A Complex Immune Phenomenon.
Reports (MDPI)Avoidance of related donors in CAEBV with germline immune variants: long-termoutcome of matched unrelated donor HSCT - a case report.
Annals of hematologySecondary hemophagocytic lymphohistiocytosis following blood transfusion in the absence of an identifiable trigger.
SAGE open medical case reportsInterferon-gamma blocking as a promising treatment for severe liver dysfunction in secondary hemophagocytic lymphohistiocytosis after liver transplantation.
JPGN reportsHemophagocytic 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 healthCytomegalovirus Infection-Associated Hemophagocytic Lymphohistiocytosis and Histiocytic Necrotising Lymphadenitis Progressing to Systemic Lupus Erythematosus: A Case Report.
CureusNavigating a Dual Hyperinflammatory State: Overlap of Drug Reaction With Eosinophilia and Systemic Symptoms (DRESS) and Secondary Hemophagocytic Lymphohistiocytosis (HLH) in a Child.
CureusAn Unusual Presentation of Secondary Hemophagocytic Lymphohistiocytosis With Melena: A Case Report.
CureusSecondary hemophagocytic lymphohistiocytosis concurrent with febrile infection-related epilepsy syndrome in a child.
SeizureCombination therapy success in refractory pediatric visceral leishmaniasis with hemophagocytic lymphohistiocytosis: A case report.
MedicineNeurological Involvement in Adult-Onset Secondary Hemophagocytic Lymphohistiocytosis: Clinical Features and Prognostic Implications.
Brain and behaviorSecondary hemophagocytic lymphohistiocytosis in patients treated with peritoneal dialysis.
Polish archives of internal medicineInfluenza B-Triggered Secondary Hemophagocytic Lymphohistiocytosis in an Adult Male: A Diagnostic Challenge.
CureusSecondary hemophagocytic lymphohistiocytosis in a patient with severe leptospirosis: a case report.
Journal of medical case reportsA case report of hemophagocytic lymphohistiocytosis occurring during ibrutinib chemotherapy with a literature review.
MedicineCoagulation dysfunction in children with secondary hemophagocytic lymphohistiocytosis: a comprehensive analysis.
Annals of medicinePresentation, Diagnosis, and Acute Treatment of Secondary Hemophagocytic Lymphohistiocytosis: A Case Report.
Cureus[Clinical Characteristics and Prognosis of Children with Hematological Malignancies Complicated by Secondary Hemophagocytic Lymphohistiocytosis].
Zhongguo shi yan xue ye xue za zhiRetrospective Multicenter Study of Human Granulocytic Anaplasmosis, France, 2012-2024.
Emerging infectious diseasesCorrigendum 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 hematologyPembrolizumab-Associated Hemophagocytic Lymphohistiocytosis in Clear Cell Renal Carcinoma: Case Report and Literature Review.
Reports (MDPI)Intravascular Large B-cell Lymphoma Presenting as Hemophagocytic Lymphohistiocytosis and Pyrexia of Unknown Origin: A Diagnostic Dilemma.
CureusAdult-Onset Still's Disease Complicated by Secondary Hemophagocytic Lymphohistiocytosis: A Case Report.
Clinical case reportsEfficacy of Etoposide-Based Therapy in Pediatric Secondary Hemophagocytic Lymphohistiocytosis.
European journal of haematologyImproving Recognition of Secondary Hemophagocytic Lymphohistiocytosis (HLH) through a Ferritin-Based Automated Alert: An Interrupted Time Series Analysis.
Clinical chemistrySecondary Hemophagocytic Lymphohistiocytosis in a Patient With Crohn's Disease Receiving Infliximab: A Diagnostic Challenge.
CureusTherapeutic Plasma Exchange in Pediatric Care: Insights from a Tertiary Care Centre Experience.
Indian pediatricsPerforin Expression and Natural Killer-Cell Proportion as Biomarkers in Secondary Hemophagocytic Lymphohistiocytosis.
Medical science monitor : international medical journal of experimental and clinical researchInvasive fusariosis after CD19 chimeric antigen receptor T-cell therapy.
ASM case reportsHistoplasmosis-Associated Hemophagocytic Lymphohistiocytosis in the Setting of Granulomatosis With Polyangiitis.
Journal of medical casesFatal 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 & cancerMonocyte/macrophages may exert prominent roles in macrophage activation syndrome.
Annals of hematologyCharacteristics of Secondary Malignancy Among Children With Primary Immunodeficiency Disorders in Saudi Arabia.
CureusAnalysis of risk factors for early death of lymphocyte subsets in adult patients with secondary hemophagocytic lymphohistiocytosis.
Experimental hematologyClinical manifestations and outcomes of EBV-related secondary hemophagocytic lymphohistiocytosis.
Annals of hematologyCase Report: Longitudinal mass cytometry profiling of a patient with disseminated histoplasmosis and secondary hemophagocytic lymphohistiocytosis.
Frontiers in immunologyPediatric Severe Eosinophilia: Etiological Spectrum, Diagnostic Algorithm, and Case-Based Insights From a Tertiary Care Center.
International journal of laboratory hematologyCytomegalovirus Viremia as A Cause of Fever of Unknown Origin in an Immunocompetent Adult.
European journal of case reports in internal medicineSecondary Hemophagocytic Lymphohistiocytosis Triggered by Subdural Empyema due to Streptococcus constellatus: A Rare Triad.
Journal of Brown hospital medicineMassive suprachoroidal hemorrhage in dengue-associated HLH: A clinical image.
IDCasesCase 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 immunologyDiagnosis and treatment of secondary hemophagocytic lymphohistiocytosis in adults.
Clinical advances in hematology & oncology : H&OLaboratory diagnosis of 44 cases of pediatric histiocytic necrotizing lymphadenitis.
Frontiers in pediatricsPrelude to the perfect storm: the many triggers of secondary hemophagocytic lymphohistiocytosis.
Postgraduate medicineImaging in Hemophagocytic Lymphohistiocytosis: A Comprehensive Pictorial Essay.
CureusSweet Syndrome-like Dermatosis as a Precursor to Overlapping Hematologic Malignancies: A Case Report and Review.
Journal of clinical medicineExploring the Complexities of Liver Transplantation for Secondary Hemophagocytic Lymphohistiocytosis-Associated Acute Liver Failure.
Pediatric transplantationHodgkin Lymphoma: An Unusual Presentation With Hemophagocytic Lymphohistiocytosis.
CureusPrognostic value of blood inflammatory composite markers in the survival of pediatric patients with secondary hemophagocytic lymphohistiocytosis.
Frontiers in pediatricsSecondary Dengue Infection Complicated by Hemophagocytic Lymphohistiocytosis: A Case Report.
Case reports in infectious diseasesA Comprehensive Guide to Typhoidal Anemia.
Prague medical reportDevelopment of an early mortality risk prediction model for pediatric patients with secondary hemophagocytic lymphohistiocytosis.
Italian journal of pediatricsSecondary 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 obstetricsCharacteristics of 21 Patients with Secondary Hemophagocytic Lymphohistiocytosis-Insights from a Single-Center Retrospective Study.
Medicina (Kaunas, Lithuania)Body mass index and hemophagocytic lymphohistiocytosis: a risk assessment in HIV-positive leukemia patients.
Annals of medicine and surgery (2012)Clinical features, course, and risk factors of infection-associated secondary hemophagocytic lymphohistiocytosis.
InfectionRuxolitinib targets JAK-STAT signaling to modulate neutrophil activation in refractory macrophage activation syndrome.
BloodSecondary hemophagocytic lymphohistiocytosis in a 5-month-old infant with IBD post-COVID-19: a case report.
Frontiers in pediatricsPerspectives on the current diagnostic and treatment paradigms in secondary hemophagocytic lymphohistiocytosis (HLH).
Orphanet journal of rare diseasesDeep insight into cytokine storm: from pathogenesis to treatment.
Signal transduction and targeted therapyRecognizing and Managing Secondary Hemophagocytic Lymphohistiocytosis in Adults: A Practical Clinical Guide.
Hematology/oncology clinics of North AmericaMacrophage Activation Syndrome as an Atypical Manifestation of Mixed Connective Tissue Disease in a 14-Year-Old Girl: A Case Report.
CureusMethylmalonic acidemia with recurrent hemophagocytic lymphohistiocytosis: a case report and review of the literature.
BMC pediatrics[Secondary hemophagocytic lymphohistiocytosis due to icteric leptospirosis with acute renal failure].
Deutsche medizinische Wochenschrift (1946)Immune Effector Cell-associated Hemophagocytic Lymphohistiocytosis-like Syndrome (IEC-HS).
Hematology/oncology clinics of North AmericaClinicopathological Prognostic Model for Survival in Adult Patients With Secondary Hemophagocytic Lymphohistiocytosis.
European journal of haematologyDisseminated Histoplasmosis with Secondary Hemophagocytic Lymphohistiocytosis in a Renal Transplant Recipient on Belatacept-based Immunosuppression.
Journal of Brown hospital medicineSevere dengue, aneurysmal sub-arachnoid hemorrhage, and hemophagocytic lymphohistiocytosis: a rare case combination.
Einstein (Sao Paulo, Brazil)Babesiosis with low parasitemia as a cause of secondary hemophagocytic lymphohistiocytosis in a previously healthy adult.
IDCasesSecondary Hemophagocytic Lymphohistiocytosis in severe COVID-19 - a retrospective cohort study.
Scientific reportsTetrandrine alleviates macrophage activation syndrome after CAR-T cell therapy.
Phytomedicine : international journal of phytotherapy and phytopharmacologyAggressive T-cell Lymphoma Smoldering As Hemophagocytic Lymphohistiocytosis: A Diagnostic and Medical Challenge.
CureusProteomic Analyses in COVID-19-Associated Secondary Hemophagocytic Lymphohistiocytosis.
Critical care explorationsIntercontinentally validated diagnostic criteria for secondary hemophagocytic lymphohistiocytosis-So welcome!
Journal of internal medicineMulticenter validation of secondary hemophagocytic lymphohistiocytosis diagnostic criteria.
Journal of internal medicinePreoperative pembrolizumab‑induced hemophagocytic lymphohistiocytosis in a patient with breast cancer: A case report.
Oncology lettersCOX Regression Analysis and Mortality Risk Prediction Model of 85 Adult Patients with Secondary Hemophagocytic Lymphohistiocytosis.
British journal of hospital medicine (London, England : 2005)PRMT5 inhibition reduces hyperinflammation in a murine model of secondary hemophagocytic lymphohistiocytosis.
Blood advancesMacrophage 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 reportsSecondary 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)Hemophagocytic Lymphohistiocytosis Secondary to Miliary Tuberculosis in a Resource-Limited Setting: A Case Report.
CureusCase report: A cyclic neutropenia patient with ELANE mutation accompanied by hemophagocytic lymphohistiocytosis.
Frontiers in immunologyA rare case of complicated typhoid fever presenting with secondary Hemophagocytic Lymphohistiocytosis.
Oxford medical case reportsTwo-Month Duration of Esophageal Button Battery Impaction in a 23-Month-Old Boy: A Case Report.
CureusA vector borne, airborne and food borne infection with secondary hemophagocytic lymphohistocytosis: Case of triple infections in an immuno-competent patient.
Indian journal of medical microbiologyHemophagocytic Lymphohistiocytosis (HLH): A Rare Complication of Dengue Hemorrhagic Fever.
CureusTreatment of Secondary Hemophagocytic Lymphohistiocytosis Associated With Diffuse Large B-cell Lymphoma Using Loncastuximab Tesirine As Lymphoma-Directed Therapy.
CureusA Rare Case of Disseminated Strongyloidiasis Leading to Multisystem Organ Failure Following Deceased Donor Kidney Transplantation.
The American surgeonEnteric Fever with Intussusception, Myocardial Dysfunction, Encephalopathy, and Hemophagocytic Lymphohistiocytosis in an Indian Child.
The American journal of tropical medicine and hygieneA 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 : JDDGCircMETTL3-156aa reshapes the glycolytic metabolism of macrophages to promote M1 polarization and induce cytokine storms in sHLH.
Cell death discoveryThe 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 MedicinePredicting 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 MedicinePeripheral blood smear reveals human granulocytic anaplasmosis, a rare cause of hemophagocytic lymphohistiocytosis.
American journal of hematologySecondary Hemophagocytic Lymphohistiocytosis Following Dostarlimab Treatment in a Patient With Metastatic Endometrial Cancer.
World journal of oncologyImmune biomarkers and secondary hemophagocytic lymphohistiocytosis: a scoping review protocol.
JBI evidence synthesisEBV-triggered secondary macrophage activation syndrome in the backdrop of lupus in an adult.
Indian journal of pathology & microbiology[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 zhiIdentification of biomarkers for COVID-19 associated secondary hemophagocytic lymphohistiocytosis.
bioRxiv : the preprint server for biologyHemophagocytic Lymphohistiocytosis Unmasking Systemic Lupus Erythematosus: Management With Belimumab and a Case Study.
CureusSecondary hemophagocytic lymphohistiocytosis triggered by Staphylococcus aureus bacteremia: A case report and systemic review.
IDCasesOther Immunomodulatory Treatment for Cytokine Storm Syndromes.
Advances in experimental medicine and biologyIL-1 Family Blockade in Cytokine Storm Syndromes.
Advances in experimental medicine and biologyEtoposide Therapy of Cytokine Storm Syndromes.
Advances in experimental medicine and biologyCytokine Storm and Sepsis-Induced Multiple Organ Dysfunction Syndrome.
Advances in experimental medicine and biologyGenetics of Acquired Cytokine Storm Syndromes : Secondary HLH Genetics.
Advances in experimental medicine and biologyHuman monocytotropic ehrlichiosis-A systematic review and analysis of the literature.
PLoS neglected tropical diseasesDisseminated tuberculosis complicated by intramuscular abscesses, meningoencephalitis, and hemophagocytic lymphohistiocytosis: a case report.
BMC infectious diseasesUnleashing the Cytokine Storm: A Case of Macrophage Activation Syndrome in Systemic Lupus Erythematosus.
CureusMacrophage Activation Syndrome in Viral Sepsis.
VirusesCRP and sCD25 help distinguish between adult-onset Still's disease and HLH.
European journal of haematologyUnmasking the Culprits: A Case of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis Presenting With Mouth Ulcers and Nosebleeds.
CureusComprehensive mapping of immune perturbations associated with secondary hemophagocytic lymphohistiocytosis.
Journal of leukocyte biologyFrom multisystem inflammatory syndrome in children to secondary hemophagocytic lymphohistiocytosis a series of misfortunate events: case report and review of literature.
Sudanese journal of paediatricsDiarrhea as the Initial Presentation in a Patient With HIV Diagnosed With Hodgkin's Lymphoma.
CureusA Retrospective Review of Secondary Hemophagocytic Lymphohistiocytosis (HLH) and Dengue-associated HLH from a Teaching Hospital in Singapore.
Clinical hematology internationalSingle center clinical analysis of macrophage activation syndrome complicating juvenile rheumatic diseases.
Pediatric rheumatology online journalManagement of hematological patients requiring emergency chemotherapy in the intensive care unit.
Intensive care medicineMalignancy-Associated Secondary Hemophagocytic Lymphohistiocytosis Mimicking an Infection: A Case Report and Review of the Literature.
CureusA Case of Secondary Hemophagocytic Lymphohistiocytosis in a Patient With T-cell Lymphoma.
CureusComprehensive evaluation of immune dysregulation in secondary hemophagocytic lymphohistiocytosis.
VirulenceMarginal Zone Lymphoma Manifesting as Macrophage Activation Syndrome: A Case Report.
Journal of investigative medicine high impact case reportsLow interleukin-10 level indicates a good prognosis in Salmonella enterica serovar typhimurium-induced pediatric hemophagocytic lymphohistiocytosis: A case report.
World journal of clinical casesA 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 ImmunologyTuberculosis-Associated Hemophagocytic Lymphohistiocytosis: Diagnostic Challenges and Determinants of Outcome.
Open forum infectious diseasesProbable secondary hemophagocytic lymphohistiocytosis manifesting as central nervous system lesions after COVID-19 vaccination: a case report.
Frontiers in neurologyRuxolitinib, IV Immunoglobulin, and High-Dose Glucocorticoids for Critically Ill Adults With Secondary Hemophagocytic Lymphohistiocytosis: A Single-Center Observational Pilot Study.
Critical care explorationsThe 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 & rheumatology (Hoboken, N.J.)Hemophagocytic Lymphohistiocytosis Associated With Human Herpesvirus-6 (HHV-6) Infection in an Immunocompetent Adult: A Case Report.
CureusSecondary hemophagocytic lymphohistiocytosis in pediatric patients with visceral leishmaniasis and Epstein-Barr virus infection.
Annals of hematologyDengue Hemorrhagic Fever Causing Postpartum Hemorrhage and Hemophagocytic Lymphohistiocytosis in a Young Woman: A Case Report.
Cureus[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)Severe Lymphoma-Associated Hemophagocytic Syndrome in a Young Woman.
CureusImmunopathology of and potential therapeutics for secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome: a translational perspective.
Experimental & molecular medicineAssociation of a decreased platelet count with poor survival in patients with adult secondary hemophagocytic lymphohistiocytosis.
Annals of hematologyValue of hemophagocytosis in the diagnosis of hemophagocytic lymphohistiocytosis in critically ill patients.
European journal of haematologyHemophagocytic lymphohistiocytosis: A scourge for the physician and bane to the bone marrow.
Annals of African medicineComparison 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 UniversityCase Report: Successful avoidance of etoposide for primary hemophagocytic lymphohistiocytosis-induced multiple organ dysfunction syndrome using emapalumab.
Frontiers in pediatricsCase Report: A Case of Complicated Typhoid Fever with Rhabdomyolysis and Hemophagocytic Lymphohistiocytosis.
The American journal of tropical medicine and hygienePerformances of the H-score and the HLH-2004 score in the positive diagnosis of secondary hemophagocytic lymphohistiocytosis.
Current research in translational medicineA 10-Year Retrospective Study on Pediatric Visceral Leishmaniasis in a European Endemic Area: Diagnostic and Short-Course Therapeutic Strategies.
Healthcare (Basel, Switzerland)Q fever, a rare cause of secondary hemophagocytic lymphohistiocytosis.
GMS infectious diseasesElevated monocyte HLA-DR in pediatric secondary hemophagocytic lymphohistiocytosis: a retrospective study.
Frontiers in immunology[Analysis of High-Risk Clinical Factors of Early Death in Secondary Hemophagocytic Lymphohistiocytosis].
Zhongguo shi yan xue ye xue za zhiFoamy Macrophages in a Case of Mononucleosis With Amoxicillin-Induced Rash, Hyperlipidemia, and Hemophagocytic Lymphohistiocytosis.
The American Journal of dermatopathologyHemophagocytic Lymphohistiocytosis: A Rare Complication of COVID-19 in a Patient With Sickle Cell Anemia.
CureusSevere Acute Liver Injury due to Secondary Hemophagocytic Lymphohistiocytosis: A Case Report.
GE Portuguese journal of gastroenterologySolid organ graft-versus-host disease in a recipient of a COVID-19 positive liver graft.
Journal of liver transplantationEpithelioid granulomas with proliferating T-lymphocytes in bone marrow in a patient with infectious mononucleosis modified by secondary hemophagocytic lymphohistiocytosis.
Pathology internationalThe signature and predictive value of immune parameters in patients with secondary hemophagocytic lymphohistiocytosis.
ImmunobiologyAn elevated HScore is associated with poor clinical outcomes in COVID-19.
Revista medica de ChileOutcome of patients with primary and secondary hemophagocytic lymphohistiocytosis: A retrospective analysis from a tertiary care center.
MedicineSecondary Hemophagocytic Lymphohistiocytosis: A Series of Three Cases.
CureusHemophagocytic lymphohistiocytosis-how common and how severe is it as a complication of malaria? Retrospective case series and review of the literature.
InfectionSevere acute herpes virus type 2 primo-infection and its association with anti-type 1 interferon autoantibodies.
European journal of clinical microbiology & infectious diseases : official publication of the European Society of Clinical MicrobiologyGenetic and clinical characteristics of primary hemophagocytic lymphohistiocytosis in children.
Annals of hematologyRare case of secondary hemophagocytic lymphohistiocytosis in a patient with disseminated histoplasmosis.
Pediatric investigationCLINICAL UTILITY OF INTERLEUKIN-1 INHIBITORS IN PEDIATRIC SEPSIS.
Shock (Augusta, Ga.)Sepsis as the Grand Mimic of Secondary Hemophagocytic Lymphohistiocytosis: Serratia marcescens Bacteremia with Concomitant Decompensated Cirrhotic Liver Disease.
Case reports in critical careEhrlichia-Induced Hemophagocytic Lymphohistiocytosis (HLH) With No Response to Doxycycline Treatment.
CureusSecondary hemophagocytic lymphohistiocytosis in children (Review).
Experimental and therapeutic medicineSecondary Hemophagocytic Lymphohistiocytosis Due to Typhoid Fever.
Cureus[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 xueAcute Liver Failure Caused by Secondary Hemophagocytic Lymphohistiocytosis After COVID-19 Vaccination - Case Report and Literature Review.
International medical case reports journalA novel prognosis-prediction model based on coagulation indicators in secondary hemophagocytic lymphohistiocytosis.
Annals of hematologySecondary hemophagocytic lymphohistiocytosis: an unusual complication in disseminated Mycobacterium tuberculosis.
Clinical medicine (London, England)CXCL9 inhibition does not ameliorate disease in murine models of both primary and secondary hemophagocytic lymphohistiocytosis.
Scientific reportsHemophagocytic inflammatory syndrome in ADA-SCID: report of two cases and literature review.
Frontiers in immunologyAnakinra versus etoposide-based therapy added to high-dose steroids for the treatment of secondary hemophagocytic lymphohistiocytosis.
European journal of haematology[Expression and Clinical Significance of Serum sFas/sFasL in Patients with Secondary Hemophagocytic Lymphohistiocytosis].
Zhongguo shi yan xue ye xue za zhiA Diagnostic Dilemma: Adult-Onset Still's Disease With Secondary Hemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome?
Journal of medical casesClinical value of soluble fms-like tyrosine kinase 1 (sFlt-1) in adult secondary hemophagocytic lymphohistiocytosis.
Journal of the Chinese Medical Association : JCMACase report: Sulfasalazine-induced hypersensitivity.
Frontiers in medicineRisk factors and prognosis of early death in secondary hemophagocytic lymphohistiocytosis.
Annals of hematologyPrognostic value of the albumin-bilirubin score in patients with non-Hodgkin lymphoma-associated hemophagocytic lymphohistiocytosis.
Frontiers in immunologySecondary hemophagocytic lymphohistiocytosis associated with heat stroke: A case report and review of literature.
MedicineA predictive model for identifying secondary underlying diseases of hemophagocytic lymphohistiocytosis.
Frontiers in immunologyPrimary Gastrointestinal Diffuse Large B-cell Lymphoma Presenting as Ileal Perforation.
CureusPresentations and outcomes of familial hemophagocytic lymphohistiocytosis in the pediatric intensive care units (PICUs).
Frontiers in pediatricsPostpartum fevers, a rare presentation of secondary hemophagocytic lymphohistiocytosis.
Clinical case reportsVariations in Macrophage Activation Syndrome-associated Cardiac Diseases: A Report on Two Cases.
Iranian journal of immunology : IJIPrognostic value of lipid profile in adult hemophagocytic lymphohistiocytosis.
Frontiers in oncologyHemophagocytic lymphohistiocytosis in gastric cancer: A rare syndrome for the oncologist. Case report and brief review.
Frontiers in oncologySevere COVID-19-Induced Hemophagocytic Lymphohistiocytosis.
CureusSevere 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 ResearchA Case of Disseminated Histoplasmosis From California, in the Setting of Secondary Hemophagocytic Lymphohistiocytosis: A Diagnostic Challenge.
Journal of investigative medicine high impact case reportsAcute 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 SurgeonsAcanthamoeba meningoencephalitis causing secondary hemophagocytic lymphohistiocytosis in an immunocompetent patient: A first case report.
The journal of the Royal College of Physicians of EdinburghDisseminated Cytomegalovirus Infection in a Child with Langerhans Cell Histiocytosis.
Journal of global infectious diseasesProlonged Thrombocytopenia in a Case of MIS-C in a Vaccinated Child.
Journal of investigative medicine high impact case reportsSecondary hemophagocytic lymphohistiocytosis due to Heartland virus.
BMJ case reportsManagement of Dengue: An Updated Review.
Indian journal of pediatricsHemophagocytic Lymphohistiocytosis in a Patient With Post-acute COVID-19 Infection.
CureusCase report: Drug reaction with eosinophilia and systemic symptoms (DRESS)-induced hemophagocytic disorder.
Frontiers in pharmacologySecondary 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 TherapySecondary hemophagocytic lymphohistiocytosis triggered by peripheral T-cell lymphoma: An unusual case report.
Clinical case reportsAssociações
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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.
- Neurological Involvement in Adult-Onset Secondary Hemophagocytic Lymphohistiocytosis: Clinical Features and Prognostic Implications.
- 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.
- A 70-Year-Old Woman Receiving Immunosuppressive Therapy Presenting With Iliopsoas Abscess, Pulmonary Miliary Tuberculosis, and Secondary Hemophagocytic Lymphohistiocytosis.
- Case Reports of Visceral Leishmaniasis-Associated Hemophagocytic Lymphohistiocytosis in Adults: A Complex Immune Phenomenon.
- Avoidance of related donors in CAEBV with germline immune variants: long-termoutcome of matched unrelated donor HSCT - a case report.
- Secondary Hemophagocytic Lymphohistiocytosis in a Rocky Mountain Spotted Fever Patient.
- Pneumococcal HUS in infancy masking DGKE-related thrombotic microangiopathy with secondary hemophagocytic lymphohistiocytosis.
- Clinical Profile and Outcome of Children with Scrub typhus: A Tertiary Care Centre Observation.
- 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.
- Secondary Hemophagocytic Lymphohistiocytosis as the Initial Presentation of Metastatic Carcinoma of Likely Colorectal Origin.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:158041(Orphanet)
- MONDO:0015542(MONDO)
- GARD:20026(GARD (NIH))
- Busca completa no PubMed(PubMed)
- 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
