Advances in understanding the pathogenesis of HLH. (Q38098232)

28 August 2017

Advances in understanding the pathogenesis of HLH. (English)

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28 August 2017

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3

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28 August 2017

G Naheed Usmani

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PubMed ID

23577835

28 August 2017

Bruce A Woda

series ordinal

2

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PubMed ID

23577835

28 August 2017

language of work or name

English

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publication date

12 April 2013

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British Journal of Haematology

28 August 2017

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28 August 2017

161

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28 August 2017

5

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28 August 2017

609-622

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Childhood acute myeloid leukemia with hemophagocytosis by the blasts and inv(8)(p11q13) with MOZ-TIF2 fusion transcripts.

28 August 2017

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

21 January 2018

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Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis

21 January 2018

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Griscelli syndrome: description of a case with Rab27A mutation

21 January 2018

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Use of rituximab in conjunction with immunosuppressive chemotherapy as a novel therapy for Epstein Barr virus-associated hemophagocytic lymphohistiocytosis

21 January 2018

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Repeated TLR9 stimulation results in macrophage activation syndrome-like disease in mice.

21 January 2018

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

21 January 2018

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

21 January 2018

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Defective cytotoxic lymphocyte degranulation in syntaxin-11 deficient familial hemophagocytic lymphohistiocytosis 4 (FHL4) patients

21 January 2018

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STXBP2 mutations in children with familial haemophagocytic lymphohistiocytosis type 5

21 January 2018

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

21 January 2018

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Cytophagic histiocytic panniculitis with fatal haemophagocytic lymphohistiocytosis in a paediatric patient with perforin gene mutation

21 January 2018

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

21 January 2018

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Epstein-Barr virus LMP1 inhibits the expression of SAP gene and upregulates Th1 cytokines in the pathogenesis of hemophagocytic syndrome

21 January 2018

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A proportion of patients with lymphoma may harbor mutations of the perforin gene.

21 January 2018

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

21 January 2018

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Understanding organ dysfunction in hemophagocytic lymphohistiocytosis

21 January 2018

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

21 January 2018

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

21 January 2018

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CNS involvement at the onset of primary hemophagocytic lymphohistiocytosis

21 January 2018

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Reactive hemophagocytic syndrome in adult systemic disease: report of twenty-six cases and literature review

21 January 2018

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Infectious triggers of hemophagocytic syndrome in children.

21 January 2018

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Characteristic immune abnormalities in hemophagocytic lymphohistiocytosis

21 January 2018

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Gene expression profiling of peripheral blood from patients with untreated new-onset systemic juvenile idiopathic arthritis reveals molecular heterogeneity that may predict macrophage activation syndrome.

21 January 2018

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Munc13-4 is essential for cytolytic granules fusion and is mutated in a form of familial hemophagocytic lymphohistiocytosis (FHL3).

21 January 2018

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Imaging characteristics of hemophagocytic lymphohistiocytosis

21 January 2018

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An exclusive case of juvenile myelomonocytic leukemia in association with Kikuchi's disease and hemophagocytic lymphohistiocytosis and a review of the literature

21 January 2018

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A spectrum of neuroradiological findings in children with haemophagocytic lymphohistiocytosis.

21 January 2018

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Natural killer cell dysfunction in patients with systemic-onset juvenile rheumatoid arthritis and macrophage activation syndrome

21 January 2018

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

21 January 2018

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Experience with hemophagocytic lymphohistiocytosis/macrophage activation syndrome at a single institution

21 January 2018

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Lymphoma-associated hemophagocytic syndrome: clinical features and treatment outcome.

21 January 2018

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Biology and treatment of familial hemophagocytic lymphohistiocytosis: importance of perforin in lymphocyte-mediated cytotoxicity and triggering of apoptosis

21 January 2018

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HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.

21 January 2018

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Success with infliximab in treating refractory hemophagocytic lymphohistiocytosis

21 January 2018

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Characterization of PRF1, STX11 and UNC13D genotype-phenotype correlations in familial hemophagocytic lymphohistiocytosis

21 January 2018

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

21 January 2018

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Systemic type Epstein-Barr virus-related lymphoproliferative diseases in children and young adults: challenges for pediatric hemato-oncologists and infectious disease specialists

21 January 2018

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Nationwide survey of hemophagocytic lymphohistiocytosis in Japan.

21 January 2018

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

21 January 2018

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An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+ T cells and interferon gamma are essential for the disorder

21 January 2018

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How I treat hemophagocytic lymphohistiocytosis

21 January 2018

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Loss of intrahepatic bile ducts: an important feature of familial hemophagocytic lymphohistiocytosis

21 January 2018

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Differential cellular targets of Epstein-Barr virus (EBV) infection between acute EBV-associated hemophagocytic lymphohistiocytosis and chronic active EBV infection

21 January 2018

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Virological and immunological characteristics of fatal virus-associated haemophagocytic syndrome (VAHS).

21 January 2018

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Treatment of hemophagocytic lymphohistiocytosis with alemtuzumab in systemic lupus erythematosus

21 January 2018

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Recognition and management of macrophage activation syndrome in juvenile arthritis

21 January 2018

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Hyperferritinemia as the diagnostic clue in life-threatening hemophagocytic lymphohistiocytosis.

21 January 2018

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Clinical characteristics of hemophagocytic lymphohistiocytosis related to Kawasaki disease

21 January 2018

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Peripheral hemophagocytosis: An early indicator of advanced systemic inflammatory response syndrome/hemophagocytic syndrome

21 January 2018

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Primary Epstein-Barr virus infection associated with Kikuchi's disease and hemophagocytic lymphohistiocytosis: a case report and review of the literature

21 January 2018

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Hemophagocytic lymphohistiocytosis preceded by Kikuchi disease in children

21 January 2018

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

21 January 2018

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Association of monoclonal expansion of Epstein-Barr virus-negative CD158a+ NK cells secreting large amounts of gamma interferon with hemophagocytic lymphohistiocytosis.

21 January 2018

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Perforin is a critical physiologic regulator of T-cell activation

21 January 2018

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Viral infections associated with haemophagocytic syndrome.

21 January 2018

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

21 January 2018

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21 January 2018

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Perforin polymorphism A91V and susceptibility to B-precursor childhood acute lymphoblastic leukemia: a report from the Children's Oncology Group

21 January 2018

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Cutaneous manifestations of hemophagocytic lymphohistiocytosis

21 January 2018

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Localization of a gene for familial hemophagocytic lymphohistiocytosis at chromosome 9q21.3-22 by homozygosity mapping

21 January 2018

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21 January 2018

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Hemophagocytic syndrome after Kawasaki disease

21 January 2018

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

21 January 2018

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Virus-associated hemophagocytic syndromeA benign histiocytic proliferation distinct from malignant histiocytosis

21 January 2018

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Aberrant maturation of mutant perforin underlies the clinical diversity of hemophagocytic lymphohistiocytosis.

21 January 2018

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Spectrum and clinical implications of syntaxin 11 gene mutations in familial haemophagocytic lymphohistiocytosis: association with disease-free remissions and haematopoietic malignancies

21 January 2018

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Spectrum, and clinical and functional implications of UNC13D mutations in familial haemophagocytic lymphohistiocytosis

21 January 2018

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Two randomized trials of canakinumab in systemic juvenile idiopathic arthritis

21 January 2018

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Novel Munc13-4 mutations in children and young adult patients with haemophagocytic lymphohistiocytosis

21 January 2018

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

21 January 2018

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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.

21 January 2018

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Linkage of familial hemophagocytic lymphohistiocytosis (FHL) type-4 to chromosome 6q24 and identification of mutations in syntaxin 11

21 January 2018

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Mutation spectrum in children with primary hemophagocytic lymphohistiocytosis: molecular and functional analyses of PRF1, UNC13D, STX11, and RAB27A

21 January 2018

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Familial hemophagocytic lymphohistiocytosis type 5 (FHL-5) is caused by mutations in Munc18-2 and impaired binding to syntaxin 11

21 January 2018

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Perforin gene defects in familial hemophagocytic lymphohistiocytosis.

21 January 2018

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

21 January 2018

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

21 January 2018

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Rapid detection of intracellular SH2D1A protein in cytotoxic lymphocytes from patients with X-linked lymphoproliferative disease and their family members.

21 January 2018

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Increased IL-16 levels in hemophagocytic lymphohistiocytosis

21 January 2018

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

21 January 2018

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Quantitative analysis of cell-free Epstein-Barr virus genome copy number in patients with EBV-associated hemophagocytic lymphohistiocytosis

21 January 2018

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Clinical significance of cloned expansion and CD5 down-regulation in Epstein-Barr Virus (EBV)-infected CD8+ T lymphocytes in EBV-associated hemophagocytic lymphohistiocytosis

21 January 2018

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Genotype-phenotype study of familial haemophagocytic lymphohistiocytosis due to perforin mutations.

21 January 2018

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Correlation between phenotypic heterogeneity and gene mutational characteristics in familial hemophagocytic lymphohistiocytosis (FHL).

21 January 2018

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Natural killer cell dysfunction is a distinguishing feature of systemic onset juvenile rheumatoid arthritis and macrophage activation syndrome

21 January 2018

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Perforin-mediated target-cell death and immune homeostasis

21 January 2018

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

21 January 2018

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

21 January 2018

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Association of IRF5 polymorphisms with susceptibility to macrophage activation syndrome in patients with juvenile idiopathic arthritis.

21 January 2018

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Characterization of Epstein-Barr virus (EBV)-infected cells in EBV-associated hemophagocytic lymphohistiocytosis in two patients with X-linked lymphoproliferative syndrome type 1 and type 2.

21 January 2018

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

21 January 2018

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Cytotoxic T-lymphocyte-associated antigen 4 gene polymorphisms in Japanese children with infection-associated hemophagocytic lymphohistiocytosis

21 January 2018

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

21 January 2018

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

21 January 2018

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

21 January 2018

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Inherited defects causing hemophagocytic lymphohistiocytic syndrome

21 January 2018

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21 January 2018

Identifiers

DOI

10.1111/BJH.12293

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28 August 2017

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PubMed ID

23577835