Published in Am J Hematol on June 01, 2007
Hypomorphic mutations in PRF1, MUNC13-4, and STXBP2 are associated with adult-onset familial HLH. Blood (2011) 2.55
Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol. Blood (2011) 2.35
What nephrologists need to know about hemophagocytic syndrome. Nat Rev Nephrol (2009) 1.17
Atypical familial hemophagocytic lymphohistiocytosis due to mutations in UNC13D and STXBP2 overlaps with primary immunodeficiency diseases. Haematologica (2010) 1.15
Familial haemophagocytic lymphohistiocytosis: advances in the genetic basis, diagnosis and management. Clin Exp Immunol (2011) 1.12
Familial hemophagocytic lymphohistiocytosis: when rare diseases shed light on immune system functioning. Front Immunol (2014) 1.00
Familial hemophagocytic lymphohistiocytosis may present during adulthood: clinical and genetic features of a small series. PLoS One (2012) 0.93
Diagnostic challenges in a child with familial hemophagocytic lymphohistiocytosis type 3 (FHLH3) presenting with fulminant neurological disease. Childs Nerv Syst (2008) 0.87
Functional assessment of perforin C2 domain mutations illustrates the critical role for calcium-dependent lipid binding in perforin cytotoxic function. Blood (2008) 0.86
Hemophagocytic Lymphohistiocytosis in Children: Pathogenesis and Treatment. Front Pediatr (2016) 0.82
Epitope specificity of memory CD8+ T cells dictates vaccination-induced mortality in LCMV-infected perforin-deficient mice. Eur J Immunol (2012) 0.81
Prognostic Factors and Outcomes of Adult-Onset Hemophagocytic Lymphohistiocytosis: A Retrospective Analysis of 34 Cases. Hematol Rep (2015) 0.80
Role of perforin-mediated cell apoptosis in murine models of infusion-induced bone marrow failure. Exp Hematol (2009) 0.79
Analyses of the PRF1 gene in individuals with hemophagocytic lymphohystiocytosis reveal the common haplotype R54C/A91V in Colombian unrelated families associated with late onset disease. J Clin Immunol (2012) 0.78
Targeted high-throughput sequencing for genetic diagnostics of hemophagocytic lymphohistiocytosis. Genome Med (2015) 0.76
Successful haploidentical stem cell transplantation for three adults with primary hemophagocytic lymphohistiocytosis. Bone Marrow Transplant (2016) 0.75
Familial Hemophagocytic Lymphohistiocytosis due to Mutation of UNC13D Gene. Indian J Hematol Blood Transfus (2015) 0.75
Exome sequencing for simultaneous mutation screening in children with hemophagocytic lymphohistiocytosis. Int J Hematol (2017) 0.75
A novel pathogenic variant in PRF1 associated with hemophagocytic lymphohistiocytosis. J Clin Immunol (2015) 0.75
HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer (2007) 16.96
Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood (2002) 3.54
Novel prognostic subgroups in childhood 11q23/MLL-rearranged acute myeloid leukemia: results of an international retrospective study. Blood (2009) 2.57
Slug, a highly conserved zinc finger transcriptional repressor, protects hematopoietic progenitor cells from radiation-induced apoptosis in vivo. Cancer Cell (2002) 2.48
Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol. Blood (2011) 2.35
Nfil3/E4bp4 is required for the development and maturation of NK cells in vivo. J Exp Med (2009) 2.26
Spontaneous improvement of hematologic abnormalities in patients having juvenile myelomonocytic leukemia with specific RAS mutations. Blood (2007) 2.23
Nationwide survey of hemophagocytic lymphohistiocytosis in Japan. Int J Hematol (2007) 1.90
Anakinra improves sensory deafness in a Japanese patient with Muckle-Wells syndrome, possibly by inhibiting the cryopyrin inflammasome. Arthritis Rheum (2008) 1.72
Ikaros dominant negative isoform (Ik6) induces IL-3-independent survival of murine pro-B lymphocytes by activating JAK-STAT and up-regulating Bcl-xl levels. Leuk Lymphoma (2008) 1.72
Identification of a gene expression signature associated with pediatric AML prognosis. Blood (2003) 1.71
Proposed guidelines for diagnosing chronic active Epstein-Barr virus infection. Am J Hematol (2005) 1.65
The diagnostic utility of exome sequencing in Joubert syndrome and related disorders. J Hum Genet (2012) 1.64
Haematopoietic stem cell transplantation in haemophagocytic lymphohistiocytosis. Br J Haematol (2005) 1.64
Sensitive quantitation of minimal residual disease in chronic myeloid leukemia using nanofluidic digital polymerase chain reaction assay. Leuk Lymphoma (2011) 1.61
Prospective 5-year follow-up of cyclosporine treatment in children with steroid-resistant nephrosis. Pediatr Nephrol (2013) 1.60
Chloroquine and inhibition of Toll-like receptor 9 protect from sepsis-induced acute kidney injury. Am J Physiol Renal Physiol (2008) 1.59
Identification of FOXP3-negative regulatory T-like (CD4(+)CD25(+)CD127(low)) cells in patients with immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome. Clin Immunol (2011) 1.57
High serum values of soluble CD154, IL-2 receptor, RANKL and osteoprotegerin in Langerhans cell histiocytosis. Pediatr Blood Cancer (2006) 1.56
Features and outcome of neonatal leukemia in Japan: experience of the Japan infant leukemia study group. Pediatr Blood Cancer (2006) 1.56
Correlation between phenotypic heterogeneity and gene mutational characteristics in familial hemophagocytic lymphohistiocytosis (FHL). Pediatr Blood Cancer (2006) 1.56
Aurora kinase A-specific T-cell receptor gene transfer redirects T lymphocytes to display effective antileukemia reactivity. Blood (2011) 1.54
Neurotoxicity and physicochemical properties of Abeta mutant peptides from cerebral amyloid angiopathy: implication for the pathogenesis of cerebral amyloid angiopathy and Alzheimer's disease. J Biol Chem (2003) 1.53
Morbidity in children with frequently relapsing nephrosis: 10-year follow-up of a randomized controlled trial. Pediatr Nephrol (2014) 1.53
Henoch-Schonlein purpura presenting duodenal involvement similar to superior mesenteric artery syndrome in a girl. Eur J Pediatr (2006) 1.51
Foot process effacement is an early marker of nephropathy in young classic Fabry patients without albuminuria. Nephron (2014) 1.50
Single dose of rituximab for refractory steroid-dependent nephrotic syndrome in children. Pediatr Nephrol (2009) 1.49
Rituximab treatment combined with methylprednisolone pulse therapy and immunosuppressants for childhood steroid-resistant nephrotic syndrome. Pediatr Nephrol (2014) 1.46
Procalcitonin as a marker of respiratory disorder in neonates. Pediatr Int (2014) 1.44
Association between serum uric acid levels and cardiometabolic risk factors among Japanese junior high school students. Circ J (2010) 1.44
Serum levels of free light chain before and after chemotherapy in primary systemic AL amyloidosis. Intern Med (2005) 1.43
The status of the GH-IGF-I axis in a child with psychosocial short stature. J Pediatr Endocrinol Metab (2003) 1.40
Frequent mutations in the GATA-1 gene in the transient myeloproliferative disorder of Down syndrome. Blood (2003) 1.40
Constitutional pericentric inversion of chromosome 9 and haemopoietic stem cell transplantation: delayed engraftment. Br J Haematol (2002) 1.40
Two-step transplantation for primary hyperoxaluria: a winning strategy to prevent progression of systemic oxalosis in early onset renal insufficiency cases. Pediatr Transplant (2014) 1.40
Suppressive oligodeoxynucleotides delay the onset of glomerulonephritis and prolong survival in lupus-prone NZB x NZW mice. Arthritis Rheum (2005) 1.40
Pineal dysfunction (low melatonin production) as a cause of sudden death in a long-term survivor of Langerhans cell histiocytosis? Med Pediatr Oncol (2003) 1.39
Superior mesenteric artery syndrome: risk factor for duodenal involvement in Henoch-Schönlein purpura. Pediatr Int (2011) 1.39
Right-to-left shunting in the ductus arteriosus is induced readily by intense crying and rapid postural change in neonates with meconium-stained amniotic fluid. Pediatr Crit Care Med (2012) 1.38
Genetic subtypes of familial hemophagocytic lymphohistiocytosis: correlations with clinical features and cytotoxic T lymphocyte/natural killer cell functions. Blood (2005) 1.37
Prognostic factors for chronic active Epstein-Barr virus infection. J Infect Dis (2003) 1.36
Autoimmune lymphoproliferative syndrome-like disease with somatic KRAS mutation. Blood (2010) 1.32
Age, gender, and body length effects on reference serum creatinine levels determined by an enzymatic method in Japanese children: a multicenter study. Clin Exp Nephrol (2011) 1.31
Treatment of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH) in young adults: a report from the HLH study center. Med Pediatr Oncol (2003) 1.28
Frequency and spectrum of central nervous system involvement in 193 children with haemophagocytic lymphohistiocytosis. Br J Haematol (2007) 1.27
FLT3 mutations in the activation loop of tyrosine kinase domain are frequently found in infant ALL with MLL rearrangements and pediatric ALL with hyperdiploidy. Blood (2003) 1.26
Risk-stratified therapy and the intensive use of cytarabine improves the outcome in childhood acute myeloid leukemia: the AML99 trial from the Japanese Childhood AML Cooperative Study Group. J Clin Oncol (2009) 1.26
Renal cell carcinoma in dialysis patients: a single center experience. Int J Urol (2006) 1.26
Prognostic significance of additional cytogenetic aberrations in 733 de novo pediatric 11q23/MLL-rearranged AML patients: results of an international study. Blood (2011) 1.25
Frequent co-expression of HoxA9 and Meis1 genes in infant acute lymphoblastic leukaemia with MLL rearrangement. Br J Haematol (2002) 1.22
Perforin gene mutations in adult-onset hemophagocytic lymphohistiocytosis. Haematologica (2007) 1.21
Synthesis, aggregation, neurotoxicity, and secondary structure of various A beta 1-42 mutants of familial Alzheimer's disease at positions 21-23. Biochem Biophys Res Commun (2002) 1.20
Success with infliximab in treating refractory hemophagocytic lymphohistiocytosis. Am J Hematol (2006) 1.19
Infant acute lymphoblastic leukemia with MLL gene rearrangements: outcome following intensive chemotherapy and hematopoietic stem cell transplantation. Blood (2004) 1.18
IL-9 enhances the growth of human mast cell progenitors under stimulation with stem cell factor. J Immunol (2003) 1.17
Two distinct gene expression signatures in pediatric acute lymphoblastic leukemia with MLL rearrangements. Cancer Res (2003) 1.16
ASC, which is composed of a PYD and a CARD, is up-regulated by inflammation and apoptosis in human neutrophils. Biochem Biophys Res Commun (2002) 1.16
Single infusion of rituximab for persistent steroid-dependent minimal-change nephrotic syndrome after long-term cyclosporine. Pediatr Nephrol (2010) 1.15
Conversion of one-step to two-step self-etch adhesives for improved efficacy and extended application. Am J Dent (2005) 1.15
Occurrence of haemophagocytic lymphohistiocytosis at less than 1 year of age: analysis of 96 patients. Eur J Pediatr (2005) 1.14
Characteristic perforin gene mutations of haemophagocytic lymphohistiocytosis patients in Japan. Br J Haematol (2003) 1.14
Atypical Pneumocystis jiroveci pneumonia with multiple nodular granulomas after rituximab for refractory nephrotic syndrome. Pediatr Nephrol (2012) 1.12
Hematopoietic stem cell transplantation for familial hemophagocytic lymphohistiocytosis and Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in Japan. Pediatr Blood Cancer (2010) 1.11
Microtensile dentin bond strength of self-etching resins: effect of a hydrophobic layer. Oper Dent (2005) 1.11
Creatinine-based equation to estimate the glomerular filtration rate in Japanese children and adolescents with chronic kidney disease. Clin Exp Nephrol (2013) 1.10
Review of hemophagocytic lymphohistiocytosis (HLH) in children with focus on Japanese experiences. Crit Rev Oncol Hematol (2005) 1.09
Correlation of clinical features with the mutational status of GM-CSF signaling pathway-related genes in juvenile myelomonocytic leukemia. Pediatr Res (2009) 1.08
Mutations in the ribosomal protein genes in Japanese patients with Diamond-Blackfan anemia. Haematologica (2010) 1.07
CpG oligodeoxynucleotides protect newborn mice from a lethal challenge with the neurotropic Tacaribe arenavirus. J Immunol (2006) 1.06
Longitudinal follow-up of patients with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis. Haematologica (2004) 1.04
HNF1B alterations associated with congenital anomalies of the kidney and urinary tract. Pediatr Nephrol (2010) 1.03
Prospective study of a pirarubicin, intermediate-dose cytarabine, and etoposide regimen in children with Down syndrome and acute myeloid leukemia: the Japanese Childhood AML Cooperative Study Group. J Clin Oncol (2007) 1.03
Perforin defects of primary haemophagocytic lymphohistiocytosis in Japan. Br J Haematol (2002) 1.01
Clinical and genetic characteristics of XIAP deficiency in Japan. J Clin Immunol (2012) 1.00