Advances in understanding the pathogenesis of HLH

Clinical Characteristics of Hemophagocytic Lymphohistiocytosis Related to Kawasaki Disease

(2016) Hyun Kwon Kim et al.   PEDIATRIC HEMATOLOGY AND ONCOLOGY

Inherited defects causing hemophagocytic lymphohistiocytic syndrome

(2012) Geneviève de Saint Basile et al.   Annals of the New York Academy of Sciences

Gene-expression signatures differ between different clinical forms of familial hemophagocytic lymphohistiocytosis

(2012) J. Sumegi et al.   BLOOD

Clinical analysis of macrophage activation syndrome in pediatric patients with autoimmune diseases

(2012) Cheng-I Lin et al.   CLINICAL RHEUMATOLOGY

Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment

(2012) A Ravelli et al.   GENES AND IMMUNITY

Rapid and Sustained Remission of Systemic Juvenile Idiopathic Arthritis-Associated Macrophage Activation Syndrome Through Treatment With Anakinra and Corticosteroids

(2012) Normi Bruck et al.   JCR-JOURNAL OF CLINICAL RHEUMATOLOGY

Treatment of Hemophagocytic Lymphohistiocytosis With Alemtuzumab in Systemic Lupus Erythematosus

(2012) Michael P. Keith et al.   JCR-JOURNAL OF CLINICAL RHEUMATOLOGY

CNS involvement at the onset of primary hemophagocytic lymphohistiocytosis

(2012) K. Deiva et al.   NEUROLOGY

Two Randomized Trials of Canakinumab in Systemic Juvenile Idiopathic Arthritis

(2012) Nicolino Ruperto et al.   NEW ENGLAND JOURNAL OF MEDICINE

Efficacy, safety and tolerability of tocilizumab in patients with systemic juvenile idiopathic arthritis

(2012) Shumpei Yokota et al.   Therapeutic Advances in Musculoskeletal Disease

Familial and Acquired Hemophagocytic Lymphohistiocytosis

(2011) G.E. Janka   Annual Review of Medicine

Gene expression profiling of peripheral blood mononuclear cells from children with active hemophagocytic lymphohistiocytosis

(2011) J. Sumegi et al.   BLOOD

Perforin is a critical physiologic regulator of T-cell activation

(2011) J. E. Lykens et al.   BLOOD

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

Association of IRF5 Polymorphisms with Susceptibility to Hemophagocytic Lymphohistiocytosis in Children

(2011) Masakatsu Yanagimachi et al.   JOURNAL OF CLINICAL IMMUNOLOGY

Repeated TLR9 stimulation results in macrophage activation syndrome–like disease in mice

(2011) Edward M. Behrens et al.   JOURNAL OF CLINICAL INVESTIGATION

Association of IRF5 Polymorphisms with Susceptibility to Macrophage Activation Syndrome in Patients with Juvenile Idiopathic Arthritis

(2011) M. YANAGIMACHI et al.   JOURNAL OF RHEUMATOLOGY

A novel single point mutation of the LYST gene in two siblings with different phenotypic features of Chediak Higashi syndrome

(2011) Zuhre Kaya et al.   PEDIATRIC BLOOD & CANCER

Cytotoxic T-Lymphocyte-Associated Antigen 4 Gene Polymorphisms in Japanese Children with Infection-Associated Hemophagocytic Lymphohistiocytosis

(2010) Megumi Yoshiyama et al.   ACTA HAEMATOLOGICA

Neonatal liver failure and haemophagocytic lymphohistiocytosis caused by a new perforin mutation

(2010) O Danhaive et al.   ACTA PAEDIATRICA

Clinical significance of clonality and Epstein-Barr virus infection in adult patients with hemophagocytic lymphohistiocytosis

(2010) Jae-Sook Ahn et al.   AMERICAN JOURNAL OF HEMATOLOGY

Pathology of the Liver in Familial Hemophagocytic Lymphohistiocytosis

(2010) Jey-Hsin Chen et al.   AMERICAN JOURNAL OF SURGICAL PATHOLOGY

Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency)

(2010) J. P. Schmid et al.   BLOOD

X-linked lymphoproliferative disease due to SAP/SH2D1A deficiency: a multicenter study on the manifestations, management and outcome of the disease

(2010) C. Booth et al.   BLOOD

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

STXBP2 mutations in children with familial haemophagocytic lymphohistiocytosis type 5

(2010) V. Cetica et al.   JOURNAL OF MEDICAL GENETICS

Primary Epstein-Barr Virus Infection Associated with Kikuchi's Disease and Hemophagocytic Lymphohistiocytosis: A Case Report and Review of the Literature

(2010) Hao-Yuan Lee et al.   JOURNAL OF MICROBIOLOGY IMMUNOLOGY AND INFECTION

Hemophagocytic lymphohistiocytosis with MUNC13-4 gene mutation or reduced natural killer cell function prior to onset of childhood leukemia

(2010) Tamara Y. Chang et al.   PEDIATRIC BLOOD & CANCER

Unusual functional manifestations of a novel STX11 frameshift mutation in two infants with familial hemophagocytic lymphohistiocytosis type 4 (FHL4)

(2010) Christine A. Macartney et al.   PEDIATRIC BLOOD & CANCER

Development of secondary T-cell acute lymphoblastic leukemia in a child with hemophagocytic lymphohistiocytosis

(2010) Bibi Shahin Shamsian et al.   PEDIATRIC BLOOD & CANCER

Macrophage activation syndrome/hemophagocytic lymphohistiocytosis and Kawasaki disease

(2010) Gabriele Simonini et al.   PEDIATRIC BLOOD & CANCER

Viral infections associated with haemophagocytic syndrome

(2010) Nadine Rouphael Maakaroun et al.   REVIEWS IN MEDICAL VIROLOGY

Familial Hemophagocytic Lymphohistiocytosis Type 5 (FHL-5) Is Caused by Mutations in Munc18-2 and Impaired Binding to Syntaxin 11

(2009) Udo zur Stadt et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Different NK cell-activating receptors preferentially recruit Rab27a or Munc13-4 to perforin-containing granules for cytotoxicity

(2009) S. M. Wood et al.   BLOOD

Syndromic albinism and haemophagocytosis

(2009) Rubén Berrueco et al.   BRITISH JOURNAL OF HAEMATOLOGY

Familial hemophagocytic lymphohistiocytosis: clinical and neuroradiological findings and review of the literature

(2009) Norma Decaminada et al.   CHILDS NERVOUS SYSTEM

Munc18-2 deficiency causes familial hemophagocytic lymphohistiocytosis type 5 and impairs cytotoxic granule exocytosis in patient NK cells

(2009) Marjorie Côte et al.   JOURNAL OF CLINICAL INVESTIGATION

Experience With Hemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome at a Single Institution

(2009) Abha A. Gupta et al.   JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY

Hyperferritinemia as the Diagnostic Clue in Life-Threatening Hemophagocytic Lymphohistiocytosis

(2009) S. Kerzel et al.   KLINISCHE PADIATRIE

Infection of T lymphocytes in Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in children of non-Asian origin

(2009) Karin Beutel et al.   PEDIATRIC BLOOD & CANCER

Temperature sensitivity of human perforin mutants unmasks subtotal loss of cytotoxicity, delayed FHL, and a predisposition to cancer

(2009) J. Chia et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis is associated with MUNC13-4 polymorphisms

(2008) Kejian Zhang et al.   ARTHRITIS AND RHEUMATISM

Hematopoietic cell transplantation for hemophagocytic lymphohistiocytosis: a journey of a thousand miles begins with a single (big) step

(2008) M B Jordan et al.   BONE MARROW TRANSPLANTATION

Syntaxin-11 is expressed in primary human monocytesmacrophages and acts as a negative regulator of macrophage engulfment of apoptotic cells and IgG-opsonized target cells

(2008) Shouting Zhang et al.   BRITISH JOURNAL OF HAEMATOLOGY

Characterization ofPRF1,STX11andUNC13Dgenotype-phenotype correlations in familial hemophagocytic lymphohistiocytosis

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

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

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

Diagnostic challenges in a child with familial hemophagocytic lymphohistiocytosis type 3 (FHLH3) presenting with fulminant neurological disease

(2008) James D. Weisfeld-Adams et al.   CHILDS NERVOUS SYSTEM

Association of Monoclonal Expansion of Epstein-Barr Virus-Negative CD158a+ NK Cells Secreting Large Amounts of Gamma Interferon with Hemophagocytic Lymphohistiocytosis

(2008) M. R. Lopez-Alvarez et al.   Clinical and Vaccine Immunology

Understanding organ dysfunction in hemophagocytic lymphohistiocytosis

(2008) Caroline Créput et al.   INTENSIVE CARE MEDICINE

Hemophagocytic Lymphohistiocytosis Associated With Uncontrolled Inflammatory Cytokinemia and Chemokinemia was Caused by Systemic Anaplastic Large Cell Lymphoma: A Case Report and Review of the Literature

(2008) Akira Shimada et al.   JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY

Pronounced hyperferritinemia: Expanding the field of hemophagocytic lymphohistiocytosis

(2008) Jan-Inge Henter   PEDIATRIC BLOOD & CANCER

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

High elevated ferritin levels and the diagnosis of HLH/Sepsis/SIRS/MODS/MAS

(2008) Leticia Castillo   PEDIATRIC BLOOD & CANCER

The role of the initial bone marrow aspirate in the diagnosis of hemophagocytic lymphohistiocytosis

(2008) Abha Gupta et al.   PEDIATRIC BLOOD & CANCER

Hemophagocytic lymphohistiocytosis preceded by Kikuchi disease in children

(2008) Gye-Yeon Lim et al.   PEDIATRIC RADIOLOGY

Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis

(2007) Carl E. Allen et al.   PEDIATRIC BLOOD & CANCER