Keywords: Severe fever with thrombocytopenia syndrome; Hemophagocytic lymphohistiocytosis; Central nervous system involvement; Immunohistochemistry; Autopsy; CFS; cerebrospinal fluid; CNS; central nervous system; CNS-HLH; CNS involvement of HLH; HLH; hemophagocytic
مقالات ISI (ترجمه نشده)
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Keywords: Pancytopenia; Aplastic anemia; NGS; Megaloblastic anemia; PNH; HLH;
Keywords: GC; glucocorticoid; HLH; hemophagocytic lymphohistiocytosis; ICU; intensive care unit; ID; immunosuppressive drug; IVIG; intravenous immunoglobulin; NK; natural killer;
Keywords: Primary immunodeficiency; Hematologic malignancy; Lymphoma; Leukemia; Hodgkin; AD; Autosomal dominant; ADA; Adenosine deaminase; AML; Acute myeloid leukemia; AR; Autosomal recessive; CID; combined immunodeficiency; HL; Hodgkin lymphoma; HLH; Hemophagocyti
Keywords: Primary immunodeficiency; malignancy; tumor predisposition; immune surveillance; pre-existing condition; ALL; Acute lymphoblastic leukemia; ALPS; Autoimmune lymphoproliferative syndrome; AT; Ataxia teleangiectasia; CHH; Cartilage-hair hypoplasia; CID; Com
Keywords: CT; Computed tomography; EBV; Epstein-Barr virus; ECMO; Extracorporeal membrane oxygenation; FiO2; Fraction of inspired oxygen; FUO; Fever of unknown origin; GM-CSF; Granulocyte-macrophage colony-stimulating factor; HLH; Hemophagocytic lymphohistiocytosis
Keywords: Adult; Criteria; Diagnosis; HLH; Sepsis;
Keywords: Hemophagocytic lymphohistiocytosis; HLH; Umbilical cord blood transplantation; Stem cell transplantation; Pediatric;
Keywords: primary hemophagocytic lymphohistiocytosis; macrophage activation syndrome; hemophagocytic syndrome; diagnostic score; HLH; Hemophagocytic lymphohistiocytosis; MAS; Macrophage activation syndrome; pHLH; Primary hemophagocytic lymphohistiocytosis; ROC; Rec
Keywords: ADCC; antibody dependent cellular cytotoxicity; AML; acute myeloid leukemia; CMV; cytomegalovirus; CR; complete remission; HL; Hodgkin lymphoma; HLA; human leukocyte antigen; HLH; hemophagocytic lymphohistiocytosis; KIR; killer cell inhibitory immunoglobu
Keywords: Immunology; primary immunodeficiency; whole-exome sequencing; hematopoietic stem cell transplantation; common variable immunodeficiency; severe combined immunodeficiency; hyper-IgE syndrome; AIP1; Actin-interacting protein 1; APDS; Activated PI3Kδ syndro
Keywords: Lymphoma; Hemophagocytosis; HLH;
Keywords: ADCC; antibody-dependent cell-mediated cytotoxicity; AP3; adaptor protein 3; CHS; Chediak-Higashi syndrome; CMV; cytomegalovirus; CNKD; classical natural killer cell deficiency; CrkL; chicken tumor virus number 10 regulator of kinase-like; CTL; cytotoxi
Keywords: HLH; hemophagocytic lymphohistiocytosis; ALT; alanine aminotransferase; AST; asparate aminotransferase; LDH; lactate dehydrase; IL; interleukin; TNF; tumor necrosis factor; IFN; interferon; EBV; Epstein-Barr virus; CBA; cytometric bead array; Hemophagocyt
Keywords: limfohistiocytoza hemofagocytarna; HLH; cytotoksycznoÅÄ; degranulacja; komórki NK; perforyna; Hemophagocytic lymphohistiocytosis; HLH; Cytotoxicity; Degranulation; NK cells; Perforin;
Keywords: Hemophagocytic lymphohistiocytosis; PRF1; UNC13D; immunologic tests; FHL; Familial hemophagocytic lymphohistiocytosis; HLH; Hemophagocytic lymphohistiocytosis; HSCT; Hematopoietic stem cell transplantation; MAS; Macrophage activation syndrome; NK; Natural
Keywords: APC; antigen-presenting cell; CTLs; cytotoxic TÂ cells; EBV; Epstein-Barr virus; FHL; familial hemophagocytic lymphohistiocytosis; H&E; hematoxylin and eosin; HLH; hemophagocytic lymphohistiocytosis; PBMCs; peripheral blood mononuclear cells; PBS; phospha
Keywords: BW; body weight; CCC; cubic clustering criterion; CP; crude protein; CSC; diet switch of corn silage to sugarcane and back to corn silage; DGGE; denaturing gradient gel electrophoresis; DM; dry matter; FEC; fecal excretion and composition; HLH; diet switc
Keywords: Hemophagocytic lymphohistiocytosis; HLH; Fulminant hepatic failure; FHF; Herpes simplex virus 1; HSV1;
Keywords: Hemophagocytic lymphohistiocytosis; Familial hemophagocytic lymphohistiocytosis; HLH; FHL; Perforin; XLP;
Keywords: BTK; Bruton tyrosine kinase; CGD; chronic granulomatous disease; CMC; chronic mucocutaneous candidiasis; γc; γ-common; HLH; hemophagocytic lymphohistiocytosis; HSCT; hematopoietic stem cell transplantation; ITK; IL2-inducible T cell kinase; NK; natural
Keywords: CNS; Central nervous system; CSF; Cerebrospinal fluid; EBV; Epstein-Barr virus; HLH; Hemophagocytic lymphohistiocytosis; IFT; Immunofluorescence test; L-AmB; Liposomal amphotericin B; NK; Natural killer; PCR; Polymerase chain reaction;
Keywords: CHOP; cyclophosphamide, hydroxydaunorubicin, oncovin (vincristine), and prednisone; CMV; cytomegalovirus; DLBCL; diffuse large B-cell lymphoma; EBV; Epstein-Barr virus; HLH; hemophagocytic lymphohistiocytosis; NK; natural killer; OS; overall survival; PCR
Keywords: 20; 36; AVV; atrioventricular valve; AVVR; atrioventricular valve regurgitation; BCPA; bidirectional cavopulmonary anastamosis; ED; end diastole; ES; end systole; HLH; hypoplastic left heart syndrome; IVS; interventricular septum; TV; tricuspid valve; uAV
Keywords: LTA; low-temperature ash; LMF; light mineral fraction; HMF; heavy mineral fraction; HTA; high temperature ash; YQ; Yangquan; XLT; Xiaolongtan; HLH; Huolinhe; TEs; trace elements; REEs; rare earth elements; XRD; X-ray diffraction; FE-SEM/EDX; field emissio
Synergistic defects of novo FAS and homozygous UNC13D leading to autoimmune lymphoproliferative syndrome-like disease: A 10-year-old Chinese boy case report
Keywords: ALPS; Autoimmune lymphoproliferative syndrome; FHL; familial hemophagocytic lymphohistiocytosis; HLH; hemophagocytic lymphohistiocytosis; DNTs; double-negative T cells; IFN; interferon; IL; interleukin; Treg; regulatory T cells; Th; T helper cell; Foxp3+;
Hemophagocytic lymphohistiocytosis (HLH) presenting as fever of unknown origin and acute liver failure
Keywords: HLH; Acute EBV infection; Acute liver failure; Cytokine storm;
De Novo Allergy and Immune-Mediated Disorders Following Solid-Organ Transplantation-Prevalence, Natural History, and Risk Factors
Keywords: transplantation; solid organ; liver; heart; kidney; intestine; multivisceral; allergy; autoimmunity; children; immune disorder; CNI; Calcineurin inhibitor; EBV; Epstein-Barr virus; EGID; Eosinophilic gastrointestinal disorder; HLH; Hemophagocytic lympho
The Jasmonate ZIM-domain protein gene SlJAZ2 regulates plant morphology and accelerates flower initiation in Solanum lycopersicum plants
Keywords: ZIM; zinc-finger protein expressed in inflorescence meristem; TIFY; TIF[F/Y]XG; JAZ; jasmonate-ZIM; ZML; ZIM-like; NINJA; novel interactor of JAZ; TPL; topless; TPRs; TPL-related proteins; ICE-CBF/DREB; inducer of CBF expression-DRE-c-repeat-binding facto
6-Hydroxydopamine induces autophagic flux dysfunction by impairing transcription factor EB activation and lysosomal function in dopaminergic neurons and SH-SY5Y cells
Keywords: PD; Parkinson's disease; iPSC; induced pluripotent stem cells; 6-OHDA; 6-hydroxydopamine; DAT; dopamine transporter; ROS; reactive oxygen species; TUNEL; terminal deoxynucelotidyl transferase-mediated deoxyuridine triphosphate nick end labeling; CCK-8;
Transfer of gene-corrected T cells corrects humoral and cytotoxic defects in patients with X-linked lymphoproliferative disease
Keywords: X-linked lymphoproliferative disease; T-cell gene therapy; follicular helper T cells; T-cell cytotoxicity; BV; Brilliant Violet; CTL; Cytotoxic T lymphocyte; HLH; Hemophagocytic lymphohistiocytosis; HSCT; Hematopoietic stem cell transplantation; HVS; Herp
T-cell gene therapy for perforin deficiency corrects cytotoxicity defects and prevents hemophagocytic lymphohistiocytosis manifestations
Keywords: Gene therapy; hemophagocytic lymphohistiocytosis; perforin deficiency; T cells; FHL; Familial hemophagocytic lymphohistiocytosis; GFP; Green fluorescent protein; HLH; Hemophagocytic lymphohistiocytosis; HSCT; Hematopoietic stem cell transplantation; IRES;
TRAF3IP2 mediates TWEAK/TWEAKR-induced pro-fibrotic responses in cultured cardiac fibroblasts and the heart
Keywords: Fn14; CIKS; Act1; adverse cardiac remodeling; migration; Act1; NF-kappa B Activator 1; ANP; atrial natriuretic peptide; AP-1; activator protein-1; CCL; C-C Motif Chemokine Ligand; CD; Cluster of Differentiation; CF; cardiac fibroblasts; CIKS; Connection t
Short CommunicationNeuromyelitis optica, atypical hemophagocytic lymphohistiocytosis and heterozygous perforin A91V mutation
Keywords: Neuromyelitis optica; Hemophagocytic lymphohistiocytosis; Perforin; A91V; Cytomegalovirus; Azathioprine; HLH; Hemophagocytic lymphohistiocytosis; NMO; Neuromyelitis optica; NMOSD; Neuromyelitis optica spectrum disorder; LETM; Longitudinally extensive tran
Hemophagocytic Lymphohistiocytosis Masquerading as Acute Liver Failure: A Single Center Experience
Keywords: ARDS; acute respiratory distress syndrome; CMV; cytomegalovirus; CSA; cyclosporine; EBV; Epstein Barr virus; HLH; hemophagocytic lymphohistiocytosis; IVIG; intravenous immunoglobulin; MAS; macrophage activation syndrome; acute liver failure; viral hepatit
Hemophagocytic lymphohistiocytosis with high serum levels of IL-18 and predominant lymphocyte activation in a neonate born to a mother with adult-onset Still's disease
Keywords: HLH; hemophagocytic lymphohistiocytosis; CTL; cytotoxic T lymphocytes; FHL; familial HLH; MAS; macrophage activation syndrome; AOSD; adult-onset Still's disease; sJIA; systemic-onset juvenile idiopathic arthritis; NK; natural killer; IL; interleukin; sIL2
Enhancing the antimicrobial activity of Sus scrofa lysozyme by N-terminal fusion of a sextuple unique homologous peptide
Keywords: SSL; Sus scrofa lysozyme; LP; longer peptide; SP; shorter peptide; HLH; helix-loop-helix; CD; circular dichroism; AFM; atomic force microscope (microscopy); Kanamycin (PubChem CID: 6032); Trifluoroacetic acid (PubChem CID: 6422); Acetonitrile (PubChem CID
Novel peptide motifs from lysozyme suppress pro-inflammatory cytokines in macrophages by antagonizing toll-like receptor and LPS-scavenging action
Keywords: hLZ; Human lysozyme; HLH; Helix-loop-helix; H1 and H2; Helix-1 and helix-2 of human lysozyme sequence; H2-S12 or H2-S13; Helix-2 extended with two β-strands or three β-strands of human lysozyme sequence; LPS; Lipopolysaccharide; TNF-α; Tumor ne
Similar but not the same: Differential diagnosis of HLH and sepsis
Keywords: Hemophagocytic syndrome; HLH; Hemophagocytic lymphohistiocytosis; Sepsis; Inflammation; Hyperferritinemia; MAS; Macrophage activation syndrome; ALT; alanine transaminase; ANC; absolute neutrophil count; aPTT; activated partial thromboplastin time; AST; as
Use of a mouse model to identify a blood biomarker for IFNγ activity in pediatric secondary hemophagocytic lymphohistiocytosis
Keywords: HLH; hemophagocytic lymphohistiocytosis; pHLH; primary hemophagocytic lymphohistiocytosis; sHLH; secondary hemophagocytic lymphohistiocytosis; IFNγ; interferon γ; TLRs; Toll-like receptors; TNFα; tumor necrosis factor α; mIFNγ; mouse IFNγ; ALT; alan
Syntaxin 8 is required for efficient lytic granule trafficking in cytotoxic T lymphocytes
Keywords: CHX; cycloheximide; CTL; Cytotoxic T lymphocytes; E:T; effector to target ratio; FHL; familial HLH; HLH; hemophagocytic lymphohistiocytosis; LDH; lactate dehydrogenase; LG; lytic granules; IS; immunological synapse; qRT-PCR; quantitative real time polymer
Chediak-Higashi syndrome: Lysosomal trafficking regulator domains regulate exocytosis of lytic granules but not cytokine secretion by natural killer cells
Keywords: Chediak-Higashi syndrome; lysosomal trafficking regulator; natural killer cell; cytotoxicity; cytotoxic lymphocyte; lysosomes; lytic granules; exocytosis; immune deficiency; ARM/HEAT domain; Armadillo/huntingtin, elongation factor 3, protein phosphatase 2
Interleukin-18 for predicting the development of macrophage activation syndrome in systemic juvenile idiopathic arthritis
Keywords: s-JIA; systemic juvenile idiopathic arthritis; MAS; macrophage activation syndrome; IL; interleukin; HLH; hemophagocytic lymphohistiocytosis; TCZ; tocilizumab; PSL; predonisolone; CRP; C-reactive protein; AOSD; adult onset Still's disease; Interleukin-1
Neonatal Hemochromatosis: Diagnostic Work-Up Based on a Series of 56 Cases of Fetal Death and Neonatal Liver Failure
Keywords: DGUOK; Deoxyguanosine kinase; EHS; Extrahepatic siderosis; ET; Exchange transfusion; GA; Gestational age; GALD; Gestational alloimmune liver disease; HLH; Hemophagocytic lymphohistiocytosis; HUS; Hemolytic uremic syndrome; IDACH; Immunohistochemistry demo
Antimicrobial peptides and proteins in the face of extremes: Lessons from archaeocins
Keywords: Antimicrobial compounds; Bacteriocin; Archaeocin; Halocin; Sulfolobicin; AMP; antimicrobial peptide/protein; Da; dalton; Hal; halocin; HLH; helix-loop-helix; ORF; open reading frame; NHE; Na+/H+ exchanger; NRPS; nonribosomal peptide synthetase; PKS; polyk
Targeted next-generation sequencing: AÂ novel diagnostic tool for primary immunodeficiencies
Keywords: Primary immunodeficiency; diagnosis; genetics; next-generation sequencing; ALPS; Autoimmune lymphoproliferative syndrome; CID; Combined immunodeficiency; HLH; Hemophagocytic lymphohistiocytosis; IUIS; International Union of Immunodeficiency Societies; NGS
Review ArticleExtra-intestinal malignancies in inflammatory bowel disease: Results of the 3rd ECCO Pathogenesis Scientific Workshop (III)
Keywords: BCC; basal cell carcinoma; CD; Crohn's disease; CIN; Cervical intra-epithelial neoplasia; COC; Combination oral contraceptive; EBV; Epstein-Barr Virus; HLH; Haemophagocytic lymphohystiocytosis; HPV; Human papilloma virus; HSTCL; Hepatosplenic T-cell lym
Diagnosing XLP1 in patients with hemophagocytic lymphohistiocytosis
Keywords: HLH; XLP1; SAP expression; 2B4 function; NK cells; FHL; Familial hemophagocytic lymphohistiocytosis; HLH; Hemophagocytic lymphohistiocytosis; MRFI; Mean relative fluorescence intensity; NK; Natural killer; PID; Primary immune deficiency; R-ADCC; Reverse a
Characterization of Crohn disease in X-linked inhibitor of apoptosis-deficient male patients and female symptomatic carriers
Keywords: Inflammatory bowel disease; Crohn disease; X-linked inhibitor of apoptosis immunodeficiency; NOD receptors; monocytes; HLH; Hemophagocytic lymphohistiocytosis; HSCT; Allogeneic hematopoietic stem cell transplantation; IBD; Inflammatory bowel disease; MCP-
Hemophagocytic lymphohistiocytosis mimicking surgical symptoms and complications: Lessons learned from four cases
Keywords: ALT; Alanine aminotransferase; AST; Aspartate aminotransferase; CRP; C-reactive protein; HLH; Hemophagocytic lymphohistiocytosis; MRI; Magnetic resonance imaging; NK-cells; Natural killer cells; RSV; Respiratory syncytial virus; SLE; Systemic lupus erythe