Abstract

An extensive evaluation flow cytometric evaluation continues of patients with autoimmune lymphoproliferative syndrome (ALPS) and their extended-family members. The evaluation is based on characterization of the expanded double negative T-cell and B-cell populations. To date, the double negative T-cells have been demonstrated to be alpha beta TcR, CD57+, HLA-DR+, and CD45RA+. The phenotypic findings in the peripheral blood have been correlated with the histologic and phenotypic findings in the lymph tissue.

  Funding Agency

Agency
National Institute of Health (NIH)
Institute
Clinical Center (CLC)
Type
Intramural Research (Z01)
Project #
1Z01CL010287-02
Application #
6103715
Study Section
Cognition and Perception Study Section (CP)
Project Start
Project End
Budget Start
Budget End
Support Year
2
Fiscal Year
1998
Total Cost
Indirect Cost

  Institution

Name
Clinical Center
Department
Type
DUNS #
City
State
Country
United States
Zip Code

  Publications

Fleisher, Thomas A (2008) The autoimmune lymphoproliferative syndrome: an experiment of nature involving lymphocyte apoptosis. Immunol Res 40:87-92
Rao, V Koneti; Dowdell, Kennichi C; Dale, Janet K et al. (2007) Pyrimethamine treatment does not ameliorate lymphoproliferation or autoimmune disease in MRL/lpr-/- mice or in patients with autoimmune lymphoproliferative syndrome. Am J Hematol 82:1049-55
Rao, V Koneti; Carrasquillo, Jorge A; Dale, Janet K et al. (2006) Fluorodeoxyglucose positron emission tomography (FDG-PET) for monitoring lymphadenopathy in the autoimmune lymphoproliferative syndrome (ALPS). Am J Hematol 81:81-5
Rao, V Koneti; Dugan, Faith; Dale, Janet K et al. (2005) Use of mycophenolate mofetil for chronic, refractory immune cytopenias in children with autoimmune lymphoproliferative syndrome. Br J Haematol 129:534-8
Fleisher, Thomas A; Oliveira, Joao B (2005) Autoimmune lymphoproliferative syndrome. Isr Med Assoc J 7:758-61
Oliveira, Joao B; Fleisher, Thomas (2004) Autoimmune lymphoproliferative syndrome. Curr Opin Allergy Clin Immunol 4:497-503
Bleesing, Jack J H; Brown, Margaret R; Novicio, Cynthia et al. (2002) A composite picture of TcR alpha/beta(+) CD4(-)CD8(-) T Cells (alpha/beta-DNTCs) in humans with autoimmune lymphoproliferative syndrome. Clin Immunol 104:21-30
Fleisher, T A; Straus, S E; Bleesing, J J (2001) A genetic disorder of lymphocyte apoptosis involving the fas pathway: the autoimmune lymphoproliferative syndrome. Curr Allergy Asthma Rep 1:534-40
Straus, S E; Jaffe, E S; Puck, J M et al. (2001) The development of lymphomas in families with autoimmune lymphoproliferative syndrome with germline Fas mutations and defective lymphocyte apoptosis. Blood 98:194-200
Lopatin, U; Yao, X; Williams, R K et al. (2001) Increases in circulating and lymphoid tissue interleukin-10 in autoimmune lymphoproliferative syndrome are associated with disease expression. Blood 97:3161-70

Showing the most recent 10 out of 13 publications