Understanding the spectrum of haemophagocytic lymphohistiocytosis: update on diagnostic challenges and therapeutic options

2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

(2016) Angelo Ravelli et al.   ANNALS OF THE RHEUMATIC DISEASES

Janus kinase inhibition lessens inflammation and ameliorates disease in murine models of hemophagocytic lymphohistiocytosis

(2016) R. Das et al.   BLOOD

Genetic predisposition to hemophagocytic lymphohistiocytosis: Report on 500 patients from the Italian registry

(2016) Valentina Cetica et al.   JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

Infection is the major trigger of hemophagocytic syndrome in adult patients treated with biological therapies

(2016) Pilar Brito-Zerón et al.   SEMINARS IN ARTHRITIS AND RHEUMATISM

Tocilizumab in systemic juvenile idiopathic arthritis in a real-world clinical setting: results from 1 year of postmarketing surveillance follow-up of 417 patients in Japan

(2015) Shumpei Yokota et al.   ANNALS OF THE RHEUMATIC DISEASES

Pathogenesis of Macrophage Activation Syndrome and Potential for Cytokine- Directed Therapies

(2015) Grant S. Schulert et al.   Annual Review of Medicine

Increased level of H-ferritin and its imbalance with L-ferritin, in bone marrow and liver of patients with adult onset Still's disease, developing macrophage activation syndrome, correlate with the severity of the disease

(2015) Piero Ruscitti et al.   AUTOIMMUNITY REVIEWS

ST2 contributes to T-cell hyperactivation and fatal hemophagocytic lymphohistiocytosis in mice

(2015) J. E. Rood et al.   BLOOD

Multicenter study of combination DEP regimen as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis

(2015) Y. Wang et al.   BLOOD

Laboratory parameters identify familial haemophagocytic lymphohistiocytosis from other forms of paediatric haemophagocytosis

(2015) Takahiro Yasumi et al.   BRITISH JOURNAL OF HAEMATOLOGY

Interleukin-18 for predicting the development of macrophage activation syndrome in systemic juvenile idiopathic arthritis

(2015) Masaki Shimizu et al.   CLINICAL IMMUNOLOGY

Hemophagocytic lymphohistiocytosis (HLH): A heterogeneous spectrum of cytokine-driven immune disorders

(2015) Ellen Brisse et al.   CYTOKINE & GROWTH FACTOR REVIEWS

The syndrome of hemophagocytic lymphohistiocytosis in primary immunodeficiencies: implications for differential diagnosis and pathogenesis

(2015) S. F. Bode et al.   HAEMATOLOGICA

Evaluating the optimal serum ferritin level to identify hemophagocytic lymphohistiocytosis in the critical care setting

(2015) Hayder Saeed et al.   INTERNATIONAL JOURNAL OF HEMATOLOGY

A Novel Model for IFN-γ–Mediated Autoinflammatory Syndromes

(2015) R. Lee Reinhardt et al.   JOURNAL OF IMMUNOLOGY

Viral etiology, clinical and laboratory features of adult hemophagocytic lymphohistiocytosis

(2015) Jinghong Chen et al.   JOURNAL OF MEDICAL VIROLOGY

Coagulation Disorders and Bleedings in Critically Ill Patients With Hemophagocytic Lymphohistiocytosis

(2015) Sandrine Valade et al.   MEDICINE

Perforin Gene Transfer Into Hematopoietic Stem Cells Improves Immune Dysregulation in Murine Models of Perforin Deficiency

(2015) Marlene Carmo et al.   MOLECULAR THERAPY

High levels of interferon-gamma (IFNγ) in macrophage activation syndrome (MAS) and CXCL9 levels as a biomarker for IFNγ production in MAS

(2015) C Bracaglia et al.   Pediatric Rheumatology

S100A12 as diagnostic tool in the differential diagnosis of sJIA associated MAS vs. hereditary or acquired HLH

(2015) D Holzinger et al.   Pediatric Rheumatology

Cytokines in systemic juvenile idiopathic arthritis and haemophagocytic lymphohistiocytosis: tipping the balance between interleukin-18 and interferon-γ

(2015) Karen Put et al.   RHEUMATOLOGY

Identification of the best cutoff points and clinical signs specific for early recognition of macrophage activation syndrome in active systemic juvenile idiopathic arthritis

(2015) Mikhail M. Kostik et al.   SEMINARS IN ARTHRITIS AND RHEUMATISM

Etiologies and Short-Term Mortality in Patients with Ultraelevated Serum Ferritin

(2015) Timothy Beer et al.   SOUTHERN MEDICAL JOURNAL

Profiling of EBV-Encoded microRNAs in EBV-Associated Hemophagocytic Lymphohistiocytosis

(2015) Chen Zhou et al.   TOHOKU JOURNAL OF EXPERIMENTAL MEDICINE

The syndrome of hemophagocytic lymphohistiocytosis in primary immunodeficiencies: implications for differential diagnosis and pathogenesis

(2015) S. F. Bode et al.   HAEMATOLOGICA

Inhibition of Natural Killer Cell Cytotoxicity by Interleukin-6: Implications for the Pathogenesis of Macrophage Activation Syndrome

(2015) Loredana Cifaldi et al.   Arthritis & Rheumatology

Rate and Clinical Presentation of Macrophage Activation Syndrome in Patients With Systemic Juvenile Idiopathic Arthritis Treated With Canakinumab

(2015) Alexei A. Grom et al.   Arthritis & Rheumatology

Hemophagocytic syndromes — An update

(2014) Gritta E. Janka et al.   BLOOD REVIEWS

Hyperinflammation, rather than hemophagocytosis, is the common link between macrophage activation syndrome and hemophagocytic lymphohistiocytosis

(2014) Lehn K. Weaver et al.   CURRENT OPINION IN RHEUMATOLOGY

Benefit and a possible risk of tocilizumab therapy for adult-onset Still’s disease accompanied by macrophage-activation syndrome

(2014) Masafumi Kobayashi et al.   Modern Rheumatology

Incidence and clinical presentation of primary hemophagocytic lymphohistiocytosis in Sweden

(2014) Marie Meeths et al.   PEDIATRIC BLOOD & CANCER

Determination of an appropriate cut-off value for ferritin in the diagnosis of hemophagocytic lymphohistiocytosis

(2014) Kai Lehmberg et al.   PEDIATRIC BLOOD & CANCER

Therapeutic Role of Anakinra, an Interleukin-1 Receptor Antagonist, in the Management of Secondary Hemophagocytic Lymphohistiocytosis/Sepsis/Multiple Organ Dysfunction/Macrophage Activating Syndrome in Critically Ill Children*

(2014) Surender Rajasekaran et al.   Pediatric Critical Care Medicine

Munc13-4 deficiency with CD5 downregulation on activated CD8+T cells

(2014) Taizo Wada et al.   PEDIATRICS INTERNATIONAL

Genetic Defects in Cytolysis in Macrophage Activation Syndrome

(2014) Mingce Zhang et al.   Current Rheumatology Reports

Performance of Current Guidelines for Diagnosis of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis

(2014) Sergio Davì et al.   Arthritis & Rheumatology

Brief Report: Alternative Activation of Laser-Captured Murine Hemophagocytes

(2014) Scott W. Canna et al.   Arthritis & Rheumatology

Development and Validation of the HScore, a Score for the Diagnosis of Reactive Hemophagocytic Syndrome

(2014) Laurence Fardet et al.   Arthritis & Rheumatology

Importance of Hyperbilurubinemia in Differentiation of Primary and Secondary Hemophagocytic Lymphohistiocytosis in Pediatric Cases

(2014) Seval Ozen et al.   Mediterranean Journal of Hematology and Infectious Diseases

Evaluation of the role of secretory sphingomyelinase and bioactive sphingolipids as biomarkers in hemophagocytic lymphohistiocytosis

(2013) Russell W. Jenkins et al.   AMERICAN JOURNAL OF HEMATOLOGY

IP-10/CXCL10 and MIG/CXCL9 as novel markers for the diagnosis of lymphoma-associated hemophagocytic syndrome

(2013) Hayato Maruoka et al.   ANNALS OF HEMATOLOGY

Mixed hematopoietic or T-cell chimerism above a minimal threshold restores perforin-dependent immune regulation in perforin-deficient mice

(2013) C. E. Terrell et al.   BLOOD

Etoposide-containing conditioning regimen reduces the occurrence of hemophagocytic lymphohistiocytosis after SCT

(2013) R Kobayashi et al.   BONE MARROW TRANSPLANTATION

Etoposide Selectively Ablates Activated T Cells To Control the Immunoregulatory Disorder Hemophagocytic Lymphohistiocytosis

(2013) T. S. Johnson et al.   JOURNAL OF IMMUNOLOGY

Differentiating Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis from Other Forms of Hemophagocytic Lymphohistiocytosis

(2013) Kai Lehmberg et al.   JOURNAL OF PEDIATRICS

Follistatin-like Protein 1 and the Ferritin/Erythrocyte Sedimentation Rate Ratio Are Potential Biomarkers for Dysregulated Gene Expression and Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis

(2013) M. Gorelik et al.   JOURNAL OF RHEUMATOLOGY

Higher-dose Anakinra Is Effective in a Case of Medically Refractory Macrophage Activation Syndrome

(2013) P. J. KAHN et al.   JOURNAL OF RHEUMATOLOGY

Adult haemophagocytic syndrome

(2013) Manuel Ramos-Casals et al.   LANCET

Cytokine profiles in children with primary Epstein-Barr virus infection

(2013) Taizo Wada et al.   PEDIATRIC BLOOD & CANCER

EVALUATION OF THE PLASMA MICRO RNA EXPRESSION LEVELS IN SECONDARY HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS

(2013) Ali Bay et al.   Mediterranean Journal of Hematology and Infectious Diseases

SAP gene transfer restores cellular and humoral immune function in a murine model of X-linked lymphoproliferative disease

(2012) C. Rivat et al.   BLOOD

Engulfment of hematopoietic stem cells caused by down-regulation of CD47 is critical in the pathogenesis of hemophagocytic lymphohistiocytosis

(2012) T. Kuriyama et al.   BLOOD

A prospective evaluation of degranulation assays in the rapid diagnosis of familial hemophagocytic syndromes

(2012) Y. T. Bryceson et al.   BLOOD

Tocilizumab masks the clinical symptoms of systemic juvenile idiopathic arthritis-associated macrophage activation syndrome: The diagnostic significance of interleukin-18 and interleukin-6

(2012) Masaki Shimizu et al.   CYTOKINE

Hemophagocytic Macrophages in Murine Typhoid Fever Have an Anti-Inflammatory Phenotype

(2012) Melissa W. McCoy et al.   INFECTION AND IMMUNITY

Protection from Inflammatory Organ Damage in a Murine Model of Hemophagocytic Lymphohistiocytosis Using Treatment with IL-18 Binding Protein

(2012) Laura Chiossone et al.   Frontiers in Immunology

How I treat hemophagocytic lymphohistiocytosis

(2011) M. B. Jordan et al.   BLOOD

Hypomorphic mutations in PRF1, MUNC13-4, and STXBP2 are associated with adult-onset familial HLH

(2011) K. Zhang et al.   BLOOD

Serum Neopterin Levels as a Diagnostic Marker of Hemophagocytic Lymphohistiocytosis Syndrome

(2011) Maria F. Ibarra et al.   Clinical and Vaccine Immunology

Not all hemophagocytes are created equally

(2011) Scott W. Canna et al.   CURRENT OPINION IN RHEUMATOLOGY

Hemophagocytosis causes a consumptive anemia of inflammation

(2011) Erin E. Zoller et al.   JOURNAL OF EXPERIMENTAL MEDICINE

An International Consensus Survey of Diagnostic Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis

(2011) S. DAVI et al.   JOURNAL OF RHEUMATOLOGY

Efficacy of peroxisome proliferator activated receptor agonist in the treatment of virus-associated haemophagocytic syndrome in a rabbit model

(2010) Wen-Chuan Hsieh et al.   ANTIVIRAL THERAPY

Anakinra as first-line disease-modifying therapy in systemic juvenile idiopathic arthritis: Report of forty-six patients from an international multicenter series

(2010) Peter A. Nigrovic et al.   ARTHRITIS AND RHEUMATISM

Clinical Significance of Cloned Expansion and CD5 Down‐Regulation in Epstein‐Barr Virus (EBV)–Infected CD8+T Lymphocytes in EBV‐Associated Hemophagocytic Lymphohistiocytosis

(2010) Akiko Toga et al.   JOURNAL OF INFECTIOUS DISEASES

Cytokine Profiles of Macrophage Activation Syndrome Associated with Rheumatic Diseases

(2010) J. MARUYAMA et al.   JOURNAL OF RHEUMATOLOGY

Failure of Interferon γ to Induce the Anti-Inflammatory Interleukin 18 Binding Protein in Familial Hemophagocytosis

(2010) Claudia A. Nold-Petry et al.   PLoS One

Distinct cytokine profiles of systemic-onset juvenile idiopathic arthritis-associated macrophage activation syndrome with particular emphasis on the role of interleukin-18 in its pathogenesis

(2010) M. Shimizu et al.   RHEUMATOLOGY

Macrophage activation syndrome in juvenile systemic lupus erythematosus: A multinational multicenter study of thirty-eight patients

(2009) Alessandro Parodi et al.   ARTHRITIS AND RHEUMATISM

Early diagnostic value of low percentage of glycosylated ferritin in secondary hemophagocytic lymphohistiocytosis

(2009) Zhao Wang et al.   INTERNATIONAL JOURNAL OF HEMATOLOGY

Hemophagocytic lymphohistiocytosis in Texas: Observations on ethnicity and race

(2009) J. Allyson Niece et al.   PEDIATRIC BLOOD & CANCER

Secondary hemophagocytic lymphohistiocytosis and severe sepsis/systemic inflammatory response syndrome/multiorgan dysfunction syndrome/macrophage activation syndrome share common intermediate phenotypes on a spectrum of inflammation

(2009) Leticia Castillo et al.   Pediatric Critical Care Medicine

Successful use of the anti-CD25 antibody daclizumab in an adult patient with hemophagocytic lymphohistiocytosis

(2008) Rebecca L. Olin et al.   AMERICAN JOURNAL OF HEMATOLOGY

Frequency and spectrum of central nervous system involvement in 193 children with haemophagocytic lymphohistiocytosis

(2008) AnnaCarin Horne et al.   BRITISH JOURNAL OF HAEMATOLOGY

Understanding organ dysfunction in hemophagocytic lymphohistiocytosis

(2008) Caroline Créput et al.   INTENSIVE CARE MEDICINE

Increased serum monocyte chemoattractant protein-1, macrophage inflammatory protein-1β, and interleukin-8 concentrations in hemophagocytic lymphohistiocytosis

(2008) Kazushi Tamura et al.   PEDIATRIC BLOOD & CANCER