V Koneti Rao

Summary

Affiliation: National Institutes of Health
Country: USA

Publications

  1. pmc Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS): report from the 2009 NIH International Workshop
    Joao B Oliveira
    Department of Laboratory Medicine, Clinical Center, National Institutes of Health, Bethesda, MD 20892, USA
    Blood 116:e35-40. 2010
  2. pmc Autoimmune lymphoproliferative syndrome misdiagnosed as hemophagocytic lymphohistiocytosis
    Amanda Rudman Spergel
    ALPS Unit, LI NIAID, National Institutes of Health, DHHS, Room 12C106, Building 10, 10 Center Dr, Bethesda, MD 20892 1899
    Pediatrics 132:e1440-4. 2013
  3. pmc 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
  4. pmc Somatic FAS mutations are common in patients with genetically undefined autoimmune lymphoproliferative syndrome
    Kennichi 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
  5. pmc 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
  6. pmc Natural history of autoimmune lymphoproliferative syndrome associated with FAS gene mutations
    Susan Price
    Molecular Development Section, Laboratory of Immunology, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD
    Blood 123:1989-99. 2014
  7. pmc Characterization and treatment of chronic active Epstein-Barr virus disease: a 28-year experience in the United States
    Jeffrey 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
  8. ncbi request reprint Pyrimethamine treatment does not ameliorate lymphoproliferation or autoimmune disease in MRL/lpr-/- mice or in patients with autoimmune lymphoproliferative syndrome
    V 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
  9. ncbi request reprint 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
  10. doi request reprint Autoimmune lymphoproliferative syndrome due to FAS mutations outside the signal-transducing death domain: molecular mechanisms and clinical penetrance
    Amy 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

Collaborators

Detail Information

Publications22

  1. pmc Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS): report from the 2009 NIH International Workshop
    Joao 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...
  2. pmc Autoimmune lymphoproliferative syndrome misdiagnosed as hemophagocytic lymphohistiocytosis
    Amanda Rudman Spergel
    ALPS Unit, LI NIAID, National Institutes of Health, DHHS, Room 12C106, Building 10, 10 Center Dr, Bethesda, MD 20892 1899
    Pediatrics 132:e1440-4. 2013
    ....
  3. pmc 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
    ....
  4. pmc Somatic FAS mutations are common in patients with genetically undefined autoimmune lymphoproliferative syndrome
    Kennichi 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...
  5. pmc 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...
  6. pmc Natural history of autoimmune lymphoproliferative syndrome associated with FAS gene mutations
    Susan Price
    Molecular Development Section, Laboratory of Immunology, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD
    Blood 123:1989-99. 2014
    ..Avoiding splenectomy while controlling hypersplenism by using corticosteroid-sparing treatments improves the outcome in ALPS-FAS patients. This trial was registered at www.clinicaltrials.gov as #NCT00001350. ..
  7. pmc Characterization and treatment of chronic active Epstein-Barr virus disease: a 28-year experience in the United States
    Jeffrey 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...
  8. ncbi request reprint Pyrimethamine treatment does not ameliorate lymphoproliferation or autoimmune disease in MRL/lpr-/- mice or in patients with autoimmune lymphoproliferative syndrome
    V 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...
  9. ncbi request reprint 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
    ....
  10. doi request reprint Autoimmune lymphoproliferative syndrome due to FAS mutations outside the signal-transducing death domain: molecular mechanisms and clinical penetrance
    Amy 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
    ....
  11. pmc FAS haploinsufficiency is a common disease mechanism in the human autoimmune lymphoproliferative syndrome
    Hye 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...
  12. pmc Somatic KRAS mutations associated with a human nonmalignant syndrome of autoimmunity and abnormal leukocyte homeostasis
    Julie 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...
  13. ncbi request reprint Use of mycophenolate mofetil for chronic, refractory immune cytopenias in children with autoimmune lymphoproliferative syndrome
    V 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...
  14. pmc A rapid ex vivo clinical diagnostic assay for fas receptor-induced T lymphocyte apoptosis
    Bernice Lo
    Laboratory of Immunology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA
    J Clin Immunol 33:479-88. 2013
    ..Hence, this rapid apoptosis assay can expedite the diagnosis of new ALPS patients, including those with somatic mutations, and facilitate clinical and molecular investigation of these diseases...
  15. pmc ALPS-ten lessons from an international workshop on a genetic disease of apoptosis
    Michael 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...
  16. pmc Development of disseminated histiocytic sarcoma in a patient with autoimmune lymphoproliferative syndrome and associated Rosai-Dorfman disease
    Girish 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...
  17. pmc How I treat autoimmune lymphoproliferative syndrome
    V 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...
  18. ncbi request reprint Characterization of ABCG2 gene amplification manifesting as extrachromosomal DNA in mitoxantrone-selected SF295 human glioblastoma cells
    V 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...
  19. ncbi request reprint Causes and consequences of the autoimmune lymphoproliferative syndrome
    V 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...
  20. doi request reprint Dominant-activating germline mutations in the gene encoding the PI(3)K catalytic subunit p110δ result in T cell senescence and human immunodeficiency
    Carrie L Lucas
    1 Molecular Development of the Immune System Section, Laboratory of Immunology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland, USA 2
    Nat Immunol 15:88-97. 2014
    ..Notably, treatment with rapamycin to inhibit mTOR activity in vivo partially restored the abundance of naive T cells, largely 'rescued' the in vitro T cell defects and improved the clinical course. ..
  21. pmc ITP: hematology's Cosette from Les Misérables
    V Koneti Rao
    National Institutes of Health, USA
    Blood 121:1928-30. 2013
    ....
  22. ncbi request reprint The extent of chromosomal aberrations induced by chemotherapy in non-human primates depends on the schedule of administration
    V 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...