(Q37729056)

22 August 2017

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title

Primary and secondary hemophagocytic lymphohistiocytosis: clinical features, pathogenesis and therapy (English)

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hemophagocytic lymphohistiocytosis

22 August 2017

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Sumit Gupta

1

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PubMed ID

20383897

22 August 2017

Sheila Weitzman

series ordinal

2

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22 August 2017

1 January 2010

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Expert Review of Clinical Immunology

22 August 2017

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22 August 2017

6

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22 August 2017

1

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22 August 2017

137-154

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Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis

22 August 2017

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Familial haemophagocytic reticulosis

21 January 2018

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Pathogen recognition and innate immunity

21 January 2018

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The biology of human natural killer-cell subsets

21 January 2018

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Relationship between CD107a expression and cytotoxic activity

21 January 2018

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Natural killer cells in antiviral defense: function and regulation by innate cytokines

21 January 2018

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Recognition of microorganisms and activation of the immune response

21 January 2018

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CD8(+) T-cell homeostasis after infection: setting the 'curve'.

21 January 2018

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Defective cytotoxic granule-mediated cell death pathway impairs T lymphocyte homeostasis

21 January 2018

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Hemophagocytic lymphohistiocytosis: diagnosis, pathophysiology, treatment, and future perspectives

21 January 2018

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Secretory mechanisms in cell-mediated cytotoxicity

21 January 2018

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Munc13-4 is an effector of rab27a and controls secretion of lysosomes in hematopoietic cells

21 January 2018

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Regulation of secretory vesicle traffic by Rab small GTPases

21 January 2018

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Hemophagocytic lymphohistiocytosis and related disorders

21 January 2018

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Delivering the kiss of death: progress on understanding how perforin works

21 January 2018

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Perforin: structure and function

21 January 2018

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Functional assessment of perforin C2 domain mutations illustrates the critical role for calcium-dependent lipid binding in perforin cytotoxic function

21 January 2018

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Lymphocyte apoptosis: refining the paths to perdition

21 January 2018

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Cytotoxic cell granule-mediated apoptosis: perforin delivers granzyme B-serglycin complexes into target cells without plasma membrane pore formation

21 January 2018

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Perforin-mediated target-cell death and immune homeostasis

21 January 2018

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The membrane repair response masks membrane disturbances caused by cell-penetrating peptide uptake

21 January 2018

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Perforin triggers a plasma membrane-repair response that facilitates CTL induction of apoptosis

21 January 2018

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21 January 2018

Granzymes at a glance

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Natural killer cell-derived human granzyme H induces an alternative, caspase-independent cell-death program

21 January 2018

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Differential expression of human granzymes A, B, and K in natural killer cells and during CD8+ T cell differentiation in peripheral blood

21 January 2018

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Interferon gamma selectively inhibits very primitive CD342+CD38- and not more mature CD34+CD38+ human hematopoietic progenitor cells

21 January 2018

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Activated cytotoxic T cells and NK cells in severe sepsis and septic shock and their role in multiple organ dysfunction

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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Analysis of natural killer-cell function in familial hemophagocytic lymphohistiocytosis (FHL): defective CD107a surface expression heralds Munc13-4 defect and discriminates between genetic subtypes of the disease

21 January 2018

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Heterogeneity of immune markers in hemophagocytic lymphohistiocytosis: comparative study of 9 familial and 14 familial inheritance-unproved cases.

21 January 2018

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Serum Levels of Interferon-gamma, Cytotoxic Factor and Soluble Interleukin-2 Receptor in Childhood Hemophagocytic Syndromes.

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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Defective natural killer cell function in patients with hemophagocytic lymphohistiocytosis and in first degree relatives

21 January 2018

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Natural killer deficiency: a minor or major factor in the manifestation of hemophagocytic lymphohistiocytosis?

21 January 2018

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Pathogenesis of hemophagocytic syndrome (HPS).

21 January 2018

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CD8+ T cell contraction is controlled by early inflammation.

21 January 2018

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The early kinetics of cytomegalovirus-specific CD8+ T-cell responses are not affected by antigen load or the absence of perforin or gamma interferon

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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Perforin gene defects in familial hemophagocytic lymphohistiocytosis.

21 January 2018

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Characteristic perforin gene mutations of haemophagocytic lymphohistiocytosis patients in Japan

21 January 2018

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Characterisation of diverse PRF1 mutations leading to decreased natural killer cell activity in North American families with haemophagocytic lymphohistiocytosis

21 January 2018

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The ABCs of granule-mediated cytotoxicity: new weapons in the arsenal

21 January 2018

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Six novel mutations in the PRF1 gene in children with haemophagocytic 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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Perforin activity and immune homeostasis: the common A91V polymorphism in perforin results in both presynaptic and postsynaptic defects in function

21 January 2018

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Linkage of familial hemophagocytic lymphohistiocytosis to 10q21-22 and evidence for heterogeneity

21 January 2018

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Further evidence for genetic heterogeneity in familial hemophagocytic lymphohistiocytosis (FHLH).

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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Mutations in RAB27A cause Griscelli syndrome associated with haemophagocytic syndrome

21 January 2018

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Review of hemophagocytic lymphohistiocytosis (HLH) in children with focus on Japanese experiences

21 January 2018

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Mutations affecting mRNA splicing are the most common molecular defect in patients with familial hemophagocytic lymphohistiocytosis type 3

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

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

21 January 2018

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Jinx, an MCMV susceptibility phenotype caused by disruption of Unc13d: a mouse model of type 3 familial hemophagocytic lymphohistiocytosis

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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Mutations of syntaxin 11 and SNAP23 genes as causes of familial hemophagocytic lymphohistiocytosis were not found in Japanese people

21 January 2018

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21 January 2018

Chediak-Higashi syndrome

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The SAP and SLAM families in immune responses and X-linked lymphoproliferative disease

21 January 2018

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Haemophagocytic lymphohistiocytosis in X-linked severe combined immunodeficiency

21 January 2018

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Familial hemophagocytic lymphohistiocytosis in two brothers with X-linked agammaglobulinemia.

21 January 2018

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Linking albinism and immunity: the secrets of secretory lysosomes

21 January 2018

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A Griscelli syndrome type 2 murine model of hemophagocytic lymphohistiocytosis (HLH).

21 January 2018

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The immunological synapse of CTL contains a secretory domain and membrane bridges.

21 January 2018

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Identification and mutation analysis of the complete gene for Chediak-Higashi syndrome

21 January 2018

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Regulation of cellular and humoral immune responses by the SLAM and SAP families of molecules.

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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XIAP deficiency in humans causes an X-linked lymphoproliferative syndrome

21 January 2018

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Haemophagocytic lymphohistiocytosis: experience at two U.K. centres

21 January 2018

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Cutaneous manifestations of 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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Fulminant neonatal liver failure in siblings: probable congenital hemophagocytic lymphohistiocytosis.

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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A spectrum of neuroradiological findings in children with haemophagocytic lymphohistiocytosis.

21 January 2018

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Natural killer cell function and interferon production in familial hemophagocytic lymphohistiocytosis

21 January 2018

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Pathogenesis of haemophagocytic lymphohistiocytosis

21 January 2018

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Modern management of children with haemophagocytic lymphohistiocytosis

21 January 2018

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

21 January 2018

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Hemophagocytic syndrome in children: an important diagnostic consideration in fever of unknown origin

21 January 2018

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Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis

21 January 2018

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CD14(dim)/CD16(bright) monocytes in hemophagocytic lymphohistiocytosis

21 January 2018

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Soluble hemoglobin-haptoglobin scavenger receptor CD163 as a lineage-specific marker in the reactive hemophagocytic syndrome.

21 January 2018

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Soluble CD163 from activated macrophages predicts mortality in acute liver failure

21 January 2018

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Nationwide survey of hemophagocytic lymphohistiocytosis in Japan.

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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Infections associated with haemophagocytic syndrome.

21 January 2018

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Cytotoxic therapy for severe avian influenza A (H5N1) infection.

21 January 2018

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Cell type specific infection of Epstein-Barr virus (EBV) in EBV-associated hemophagocytic lymphohistiocytosis and chronic active EBV infection

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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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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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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Immunophenotypic analysis of Epstein-Barr virus (EBV)-infected CD8(+) T cells in a patient with EBV-associated hemophagocytic lymphohistiocytosis

21 January 2018

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Infection- and malignancy-associated hemophagocytic syndromes. Secondary hemophagocytic lymphohistiocytosis

21 January 2018

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Precursor B-cell acute lymphoblastic leukemia presenting with hemophagocytic lymphohistiocytosis

21 January 2018

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Epstein-Barr virus associated hemophagocytic lymphohystiocytosis during maintenance treatment of acute lymphoblastic leukemia

21 January 2018

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Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders

21 January 2018

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Macrophage activation syndrome as the presenting manifestation of rheumatic diseases in childhood.

21 January 2018

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Reactive haemophagocytic syndrome in children with inflammatory disorders. A retrospective study of 24 patients

21 January 2018

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Macrophage activation syndrome and reactive hemophagocytic lymphohistiocytosis: the same entities?

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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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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Macrophage activation syndrome in rheumatic disease: what is the role of the antigen presenting cell?

21 January 2018

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Does systemic-onset juvenile idiopathic arthritis belong under juvenile idiopathic arthritis?

21 January 2018

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Genetic loci contributing to hemophagocytic lymphohistiocytosis do not confer susceptibility to systemic-onset juvenile idiopathic arthritis

21 January 2018

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Mutations of the hemophagocytic lymphohistiocytosis-associated gene UNC13D in a patient with systemic juvenile idiopathic arthritis

21 January 2018

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Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

21 January 2018

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Recognition and management of macrophage activation syndrome in juvenile arthritis

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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Successful treatment of lymphohistiocytic reticulosis with phagocytosis with epipodophyllotoxin VP 16-213.

21 January 2018

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Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation

21 January 2018

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Treatment of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) in young adults: a report from the HLH study center

21 January 2018

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Hematopoietic stem cell transplantation in hemophagocytic lymphohistiocytosis: a single-center report of 48 patients

21 January 2018

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Stem cell transplantation with reduced-intensity conditioning for hemophagocytic lymphohistiocytosis

21 January 2018

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Immunomodulation treatment for childhood virus-associated haemophagocytic lymphohistiocytosis

21 January 2018

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Management of severe neutropenia with cyclosporin during initial treatment of Epstein-Barr virus-related hemophagocytic lymphohistiocytosis

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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Macrophage activation syndrome in a patient with systemic onset rheumatoid arthritis: rescue with intravenous immunoglobulin therapy.

21 January 2018

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A case of macrophage activation syndrome successfully treated with anakinra

21 January 2018

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The role of infections in primary hemophagocytic lymphohistiocytosis: a case series and review of the literature

21 January 2018

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Recent developments in the management of haemophagocytic lymphohistiocytosis

21 January 2018

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Success with infliximab in treating refractory hemophagocytic lymphohistiocytosis

21 January 2018

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

21 January 2018

1 reference

Familial and acquired hemophagocytic lymphohistiocytosis

21 January 2018

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Immunotherapy of Familial Hemophagocytic Lymphohistiocytosis With Antithymocyte Globulins: A Single-Center Retrospective Report of 38 Patients

21 January 2018

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Innate immunity defects in Hermansky-Pudlak type 2 syndrome

21 January 2018

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Macrophage serum markers in pneumococcal bacteremia: Prediction of survival by soluble CD163*

21 January 2018

1 reference

21 January 2018

Identifiers

DOI

10.1586/ECI.09.58

1 reference

22 August 2017

1 reference

PubMed ID

20383897