Hemophagocytic syndromes

HLH‐2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis

2006 • 4,636 citations

Perforin Gene Defects in Familial Hemophagocytic Lymphohistiocytosis

1999 • 1,212 citations

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

1979 • 1,062 citations

Mutations in RAB27A cause Griscelli syndrome associated with haemophagocytic syndrome

2000 • 935 citations

Munc13-4 Is Essential for Cytolytic Granules Fusion and Is Mutated in a Form of Familial Hemophagocytic Lymphohistiocytosis (FHL3)

2003 • 905 citations

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

2002 • 836 citations

Host response to EBV infection in X-linked lymphoproliferative disease results from mutations in an SH2-domain encoding gene

1998 • 759 citations

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

2004 • 666 citations

Familial hemophagocytic lymphohistiocytosis

1983 • 567 citations

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

2005 • 552 citations

Hemophagocytic Syndromes and Infection

2000 • 552 citations

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

1996 • 536 citations

Familial and acquired hemophagocytic lymphohistiocytosis

2006 • 520 citations

Hypercytokinemia in familial hemophagocytic lymphohistiocytosis

1991 • 498 citations

Hemophagocytic lymphohistiocytosis. Report of 122 children from the International Registry. FHL Study Group of the Histiocyte Society.

1996 • 476 citations

INFECTION- AND MALIGNANCY-ASSOCIATED HEMOPHAGOCYTIC SYNDROMES

1998 • 453 citations

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

2005 • 408 citations

Linking Albinism and Immunity: The Secrets of Secretory Lysosomes

2004 • 361 citations

Macrophage activation syndrome

2002 • 343 citations

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

2001 • 329 citations

Fulminant EBV+ T-cell lymphoproliferative disorder following acute/chronic EBV infection: a distinct clinicopathologic syndrome

2000 • 290 citations

Malignant lymphoma and erythrophagocytosis simulating malignant histiocytosis

1983 • 285 citations

FAMILIAL HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS

1998 • 281 citations

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

2003 • 277 citations

Mutation spectrum in children with primary hemophagocytic lymphohistiocytosis: molecular and functional analyses ofPRF1, UNC13D, STX11, andRAB27A

2005 • 272 citations

Requirement for Etoposide in the Treatment of Epstein-Barr Virus–Associated Hemophagocytic Lymphohistiocytosis

2001 • 269 citations

Acute hemorrhagic, hepatic, and neurologic manifestations in juvenile rheumatoid arthritis: Possible relationship to drugs or infection

1985 • 246 citations

Efficacy of cyclosporine A in the treatment of macrophage activation syndrome in juvenile arthritis: Report of five cases

1996 • 243 citations

Haematopoietic stem cell transplantation in haemophagocytic lymphohistiocytosis

2005 • 227 citations

Peripheral T-cell lymphoma associated with hemophagocytic syndrome [see comments]

1990 • 227 citations

Localization of a Gene for Familial Hemophagocytic Lymphohistiocytosis at Chromosome 9q21.3-22 by Homozygosity Mapping

1999 • 216 citations

Haemophagocytic syndromes in adults: current concepts and challenges ahead

2005 • 179 citations

Hemophagocytic lymphohistiocytosis is associated with deficiencies of cellular cytolysis but normal expression of transcripts relevant to killer-cell–induced apoptosis

2002 • 163 citations

Elevated soluble interleukin-2 receptor in childhood hemophagocytic histiocytic syndromes

1989 • 163 citations

A Clinical Analysis of 52 Adult Patients With Hemophagocytic Syndrome: The Prognostic Significance of the Underlying Diseases

2001 • 161 citations

Prognostic factors of hemophagocytic syndrome in adults: analysis of 34 cases

1997 • 159 citations

Reduced perforin expression in systemic juvenile idiopathic arthritis is restored by autologous stem-cell transplantation

2003 • 140 citations

Familial hemophagocytic lymphohistiocytosis and viral infections

1993 • 129 citations

Hemophagocytic Syndrome: A Misleading Complication of Visceral Leishmaniasis in Children—A Series of 12 Cases

2000 • 128 citations

Cerebromeningeal haemophagocytic lymphohistiocytosis

1992 • 87 citations

Hemophagocytic Syndrome Presenting as Acute Hepatic Failure in Two Infants: Clinical Overlap with Neonatal Hemochromatosis

1999 • 84 citations

Natural cytotoxicity impairment in familial haemophagocytic lymphohistiocytosis.

1988 • 83 citations

Severe and progressive encephalitis as a presenting manifestation of a novel missense perforin mutation and impaired cytolytic activity

2004 • 78 citations

Histiocytic Disorders of Children and Adults

2005 • 67 citations

MIP-1alpha expression in tissues from patients with hemophagocytic syndrome.

1999 • 46 citations

Decreased expression of signaling lymphocytic-activation molecule-associated protein (SAP) transcripts in T cells from patients with rheumatoid arthritis

2001 • 41 citations

[Lymphoma-associated hemophagocytic syndrome in Japan].

1999 • 40 citations

Serum and urine beta---2-microglobulin in hemophagocytic syndrome

1995 • 38 citations

Immunosuppression: Preliminary results of alternative maintenance therapy for familial hemophagocytic lymphohistiocytosis (FHL)

1994 • 31 citations