Affiliation: Cincinnati Children's Hospital Medical Center
- Diagnosis and manifestations of chronic graft-versus-host diseaseAlexandra H Filipovich
Division of Hematology Oncology, Cincinnati Children s Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, Ohio 45229, USA
Best Pract Res Clin Haematol 21:251-7. 2008..The expectation is that the provisional guidelines described here will be widely used and refined with additional observer experience...
- Hemophagocytic lymphohistiocytosis (HLH) and related disordersAlexandra H Filipovich
Division of Hematology Oncology, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
Hematology Am Soc Hematol Educ Program . 2009....
- The expanding spectrum of hemophagocytic lymphohistiocytosisAlexandra H Filipovich
Cincinnati Children s Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio, USA
Curr Opin Allergy Clin Immunol 11:512-6. 2011....
- National Institutes of Health consensus development project on criteria for clinical trials in chronic graft-versus-host disease: I. Diagnosis and staging working group reportAlexandra H Filipovich
Cincinnati Children s Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio 45229, USA
Biol Blood Marrow Transplant 11:945-56. 2005..It is currently recommended that systemic therapy be considered for patients who meet criteria for chronic GVHD of moderate to severe global severity...
- Hemophagocytic lymphohistiocytosis and related disordersAlexandra H Filipovich
Cincinnati Children s Hospital Medical Center, ML 7015, 3333 Burnet Avenue, Cincinnati, OH 45229, USA
Curr Opin Allergy Clin Immunol 6:410-5. 2006..Awareness has grown through the Histiocyte Society and the publication of newly-recognized genetic causes. I summarize current knowledge regarding the pathophysiology, diagnosis and treatment of hemophagocytic lymphohistiocytosis...
- Hemophagocytic lymphohistiocytosis and other hemophagocytic disordersAlexandra H Filipovich
Division of Hematology Oncology, Immunodeficiency and Histiocytosis Program, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
Immunol Allergy Clin North Am 28:293-313, viii. 2008....
- Hematopoietic cell transplantation for correction of primary immunodeficienciesAh Filipovich
Immunodeficiency and Histiocytosis Program, Division of Hematology Oncology, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229, USA
Bone Marrow Transplant 42:S49-S52. 2008..While ultimate success rates are similar, transplant-related management of children receiving unrelated grafts is considerably more complicated and prolonged than following matched sibling HCT...
- Perforin expression in cytotoxic lymphocytes from patients with hemophagocytic lymphohistiocytosis and their family membersKazuhiro Kogawa
Division of Hematology Oncology, Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
Blood 99:61-6. 2002....
- Patients of African ancestry with hemophagocytic lymphohistiocytosis share a common haplotype of PRF1 with a 50delT mutationSusan Molleran Lee
Divisions of Hematology Oncology and Human Genetics, Cincinnati Children s Hospital Medical Center and the Center for Genome Information, University of Cincinnati, Cincinnati, Ohio, USA
J Pediatr 149:134-7. 2006..Extent of haplotype sharing and variability of microsatellite alleles in 50delT-PRF1 chromosomes suggest that this mutation arose approximately 1000 to 4000 years ago and is restricted to patients of African descent...
- National Institutes of Health Consensus Development Project on Criteria for Clinical Trials in Chronic Graft-versus-Host Disease: VI. Design of Clinical Trials Working Group reportPaul J Martin
Fred Hutchinson Cancer Research Center, University of Washington School of Medicine, Seattle, Washington 98109 1024, USA, and Hopital St Louis, Paris, France
Biol Blood Marrow Transplant 12:491-505. 2006..The use of consistent standards in clinical trial designs to evaluate agents that have activity in pathogenic pathways could facilitate advances in the treatment of chronic GVHD...
- Ancillary therapy and supportive care of chronic graft-versus-host disease: national institutes of health consensus development project on criteria for clinical trials in chronic Graft-versus-host disease: V. Ancillary Therapy and Supportive Care Working Daniel Couriel
University of Texas MD Anderson Cancer Center, Houston, 77030, USA
Biol Blood Marrow Transplant 12:375-96. 2006..Optimal care of patients with chronic GVHD often requires a multidisciplinary approach...
- Toward biomarkers for chronic graft-versus-host disease: National Institutes of Health consensus development project on criteria for clinical trials in chronic graft-versus-host disease: III. Biomarker Working Group ReportKirk R Schultz
British Columbia Children s Hospital, University of British Columbia, Vancouver, British Columbia, Canada
Biol Blood Marrow Transplant 12:126-37. 2006..Both approaches have merit and should be pursued. The consistent treatment and standardized documentation needed to support biomarker studies are most likely to be satisfied in prospective clinical trials...
- Life-threatening hemophagocytic syndromes: current outcomes with hematopoietic stem cell transplantationAlexandra H Filipovich
Division of Hematology Oncology, Cincinnati Children s Hospital Medical Center, Cincinnati, OH 45229 3039, USA
Pediatr Transplant 9:87-91. 2005..Over the past 25 yr, through collaborative worldwide efforts, survival of children with HLH and related disorders has improved from 5% at 1 yr after diagnosis to greater than 50% 3-5 yr after diagnosis...
- Haematopoietic stem cell transplantation in haemophagocytic lymphohistiocytosisAnnacarin Horne
Childhood Cancer Research Unit, Institution for Woman and Child Health, Karolinska Institutet, Department of Paediatric Haematology and Oncology, Karolinska Hospital, Stockholm, Sweden
Br J Haematol 129:622-30. 2005..Patients that responded well to initial pretransplant-induction therapy fared best, but some persisting HLH activity should not automatically preclude performing SCT...
- Rapid detection of intracellular SH2D1A protein in cytotoxic lymphocytes from patients with X-linked lymphoproliferative disease and their family membersYasuhiro Tabata
Division of Hematology Oncology and Division of Human Genetics, Children s Hospital Medical Center, MLC 7015, Cincinnati, OH 45229 3039, USA
Blood 105:3066-71. 2005..Four-color flow cytometry provides diagnostic information that may speed the identification of this fatal disease, differentiating it from other causes of EBV-HLH...
- Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantationJan Inge Henter
Childhood Cancer Research Unit, Karolinska Institutet, Department of Pediatric Hematology and Oncology, Karolinska Hospital, Stockholm, Sweden
Blood 100:2367-73. 2002..The 3-year probability of survival after BMT was 62% (+/- 12%). HLH-94 is very effective, allowing BMT in most patients. Survival of children with HLH has been greatly improved...
- Emergence and compartmentalization of fatal multi-drug-resistant cytomegalovirus infection in a patient with autosomal-recessive severe combined immune deficiencySamuel C Blackman
Pediatric Residency Training Program, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio 45229 3039, USA
J Pediatr Hematol Oncol 26:601-5. 2004..Serial genotypic analyses revealed multiple UL97 and UL54 (DNA polymerase) mutations that conferred phenotypic resistance to all currently licensed systemic CMV antivirals...
- Tumor immune surveillance defect of X-linked severe combined immunodeficiency is not Epstein-Barr virus specificPeter Mustillo
Division of Infectious Diseases, Nationwide Children s Hospital, Columbus, Ohio, USA
Pediatr Blood Cancer 51:706-9. 2008....
- Adolescent presentation of x-linked lymphoproliferative diseaseSamuel L Friedlander
Ann Allergy Asthma Immunol 100:398-400. 2008
- Two novel CHS1 (LYST) mutations: clinical correlations in an infant with Chediak-Higashi syndromeWafika Zarzour
Section on Human Biochemical Genetics, Medical Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD 20892, USA
Mol Genet Metab 85:125-32. 2005..These two newly described mutations are expected to give rise to a severe phenotype and, indeed, the patient had absolutely no cytotoxicity by natural killer cells or cytotoxic lymphocytes prior to his allogeneic SCT...
- Anti-IL-5 (mepolizumab) therapy reduces eosinophil activation ex vivo and increases IL-5 and IL-5 receptor levelsMiguel L Stein
Division of Allergy and Immunology, Cincinnati Children s Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio 45229, USA
J Allergy Clin Immunol 121:1473-83, 1483.e1-4. 2008..Anti-IL-5 might be a useful therapeutic agent for eosinophilic disorders, yet its immunologic consequences have not been well characterized...
- Mutations of the hemophagocytic lymphohistiocytosis-associated gene UNC13D in a patient with systemic juvenile idiopathic arthritisMelissa M Hazen
Children s Hospital Boston, Boston, Massachusetts, USA
Arthritis Rheum 58:567-70. 2008..This case broadens the range of clinical phenotypes attributable to UNC13D mutations and offers new insights into the etiology and pathogenesis of systemic JIA...
- Frequency and spectrum of central nervous system involvement in 193 children with haemophagocytic lymphohistiocytosisAnnacarin Horne
Childhood Cancer Research Unit, Department of Woman and Child Health, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden
Br J Haematol 140:327-35. 2008..Prompt treatment of HLH at onset or relapse may reduce these complications...
- Altered dynamics of Kv1.3 channel compartmentalization in the immunological synapse in systemic lupus erythematosusStella A Nicolaou
Department of Internal Medicine, University of Cincinnati, 231 Albert Sabin Way, Cincinnati, OH 45267, USA
J Immunol 179:346-56. 2007..3 constitutes the dominant K conductance. The defective temporal and spatial Kv1.3 distribution that we observed may contribute to the abnormal functions of SLE T cells...
- Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis and X-linked lymphoproliferative disease: a mimicker of sepsis in the pediatric intensive care unitMatthew Mischler
Division of Pediatric Critical Care Medicine, C S Mott Children s Hospital, University of Michigan, Ann Arbor, Michigan 48109, USA
Pediatrics 119:e1212-8. 2007....
- Healing hemophagocytosisAlexandra H Filipovich
Center for Heritable Immune Deficiencies, Cincinnati Children s Hospital Medical Center, Cincinnati, Ohio, USA
Clin Immunol 117:121-4. 2005
- Aberrant maturation of mutant perforin underlies the clinical diversity of hemophagocytic lymphohistiocytosisKimberly A Risma
Division of Allergy Immunology and Division of Hematology Oncology, Cincinnati Children s Hospital Medical Center, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio 45229, USA
J Clin Invest 116:182-92. 2006..Thus, the pathologic mechanism of perforin missense mutation likely involves a protein dosage effect of the mature protein...
- HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosisJan Inge Henter
Childhood Cancer Research Unit, Department of Woman and Child Health, Karolinska University Hospital, Karolinska Institutet, Stockholm, Sweden
Pediatr Blood Cancer 48:124-31. 2007..In order to hopefully further improve diagnosis, therapy and biological understanding, participation in HLH studies is encouraged...