Research Topics
| V Koneti RaoSummaryAffiliation: National Institutes of Health Country: USA Publications
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Detail Information
Publications
Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS): report from the 2009 NIH International WorkshopJoao B Oliveira
Department of Laboratory Medicine, Clinical Center, National Institutes of Health, Bethesda, MD 20892, USA
Blood 116:e35-40. 2010..It is hoped that harmonizing the diagnosis and classification of ALPS will foster collaborative research and better understanding of the pathogenesis of autoimmune cytopenias and B-cell lymphomas...
Use of rituximab for refractory cytopenias associated with autoimmune lymphoproliferative syndrome (ALPS)V Koneti Rao
Laboratory of Clinical Infectious Diseases, NIAID, and Clinical Center, National Institutes of Health, Bethesda, Maryland 20892 1888, USA
Pediatr Blood Cancer 52:847-52. 2009....
Somatic FAS mutations are common in patients with genetically undefined autoimmune lymphoproliferative syndromeKennichi C Dowdell
Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892 1888, USA
Blood 115:5164-9. 2010..These findings also highlight the potential role for somatic mutations in the pathogenesis of nonmalignant and/or autoimmune hematologic conditions in adults and children...
Valproic acid (VPA), a histone deacetylase (HDAC) inhibitor, diminishes lymphoproliferation in the Fas -deficient MRL/lpr(-/-) murine model of autoimmune lymphoproliferative syndrome (ALPS)Kennichi C Dowdell
Laboratory of Clinical Infectious Diseases, National Institutes of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892 1888, USA
Exp Hematol 37:487-94. 2009..We sought to test the ability of valproic acid (VPA), a histone deacetylase inhibitor, to induce apoptosis and inhibit lymphoproliferation...
Characterization and treatment of chronic active Epstein-Barr virus disease: a 28-year experience in the United StatesJeffrey I Cohen
Laboratory of Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, 50 South Drive, Bethesda, MD 20892, USA
Blood 117:5835-49. 2011..These studies are registered at http://www.clinicaltrials.gov as NCT00032513 for CAEBV, NCT00062868 and NCT00058812 for EBV-specific T-cell studies, and NCT00578539 for the hematopoietic stem cell transplantation protocol...
Fluorodeoxyglucose positron emission tomography (FDG-PET) for monitoring lymphadenopathy in the autoimmune lymphoproliferative syndrome (ALPS)V Koneti Rao
Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland 20892-1888, USA
Am J Hematol 81:81-5. 2006....
Autoimmune lymphoproliferative syndrome due to FAS mutations outside the signal-transducing death domain: molecular mechanisms and clinical penetranceAmy P Hsu
Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland, USA
Genet Med 14:81-9. 2012....
FAS haploinsufficiency is a common disease mechanism in the human autoimmune lymphoproliferative syndromeHye Sun Kuehn
Department of Laboratory Medicine, Clinical Center, National Institutes of Health, Bethesda, MD 20814, USA
J Immunol 186:6035-43. 2011..The apoptosis defect could be corrected by FAS overexpression in vitro. Our findings define haploinsufficiency as a common disease mechanism in ALPS patients with extracellular FAS mutations...
Somatic KRAS mutations associated with a human nonmalignant syndrome of autoimmunity and abnormal leukocyte homeostasisJulie E Niemela
Department of Laboratory Medicine, Warren Grant Magnuson Clinical Center, Bethesda, MD, USA
Blood 117:2883-6. 2011..We suggest the use of the term RAS-associated autoimmune leukoproliferative disease to differentiate this disorder from autoimmune lymphoproliferative syndrome...
Use of mycophenolate mofetil for chronic, refractory immune cytopenias in children with autoimmune lymphoproliferative syndromeV Koneti Rao
Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases/NIH, 10 Center Drive, Bethesda, MD 20892, USA
Br J Haematol 129:534-8. 2005..This preliminary experience suggests that MMF may spare steroid usage in patients with ALPS-associated cytopenias...
Pyrimethamine treatment does not ameliorate lymphoproliferation or autoimmune disease in MRL/lpr-/- mice or in patients with autoimmune lymphoproliferative syndromeV Koneti Rao
Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland 20892, USA
Am J Hematol 82:1049-55. 2007..We conclude that these drugs do not warrant further use empirically or as part of clinical trials in ALPS Type Ia as a lympholytic agent...
Development of disseminated histiocytic sarcoma in a patient with autoimmune lymphoproliferative syndrome and associated Rosai-Dorfman diseaseGirish Venkataraman
Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA
Am J Surg Pathol 34:589-94. 2010..Given the central role of defective Fas signaling in ALPS, histiocytes may be yet another lineage at risk for neoplastic transformation secondary to a block in apoptosis...
ALPS-ten lessons from an international workshop on a genetic disease of apoptosisMichael J Lenardo
Molecular Development Section, Laboratory of Immunology, NIAID, National Institutes of Health, Bethesda, MD 20892, USA
Immunity 32:291-5. 2010..Their discussions yielded ten broad messages applicable to genetic and immunological investigations of human disease...
How I treat autoimmune lymphoproliferative syndromeV Koneti Rao
ALPS Unit, Laboratory of Clinical and Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health NIH, Bethesda, MD 20892, USA
Blood 118:5741-51. 2011..The best approaches to diagnosis, follow-up, and management of ALPS, its associated cytopenias, and other complications resulting from infiltrative lymphoproliferation and autoimmunity are presented...
Characterization of ABCG2 gene amplification manifesting as extrachromosomal DNA in mitoxantrone-selected SF295 human glioblastoma cellsV Koneti Rao
Center for Cancer Research, National Cancer Institute, National Institutes of Health, Building 10, Room 12C103, 9000 Rockville Pike, Bethesda, MD 20892, USA
Cancer Genet Cytogenet 160:126-33. 2005..This occurred with increasing drug-selection pressure, generating a more stable genotype...
Causes and consequences of the autoimmune lymphoproliferative syndromeV Koneti Rao
Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Building 10, Room 11 N228, 10 Center Drive, Bethesda, MD 20892 1888, USA
Hematology 11:15-23. 2006..Thus, ALPS highlights the importance of cell death pathways in health and disease...
The extent of chromosomal aberrations induced by chemotherapy in non-human primates depends on the schedule of administrationV Koneti Rao
Center for Cancer Research, National Cancer Institute, NIH, Building 10, Rm 12N226, 9000 Rockville Pike, Bethesda, MD 20892, USA
Mutat Res 583:105-19. 2005..We believe this approach provides a reproducible model in which drugs and eventually, regimens can be compared...
