Assessment of Lymphocytes in Patients with Autoimmune Lymphoproliferative Syndrom

Fleisher, Thomas A.

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+. This study has been extended to characterize the double negative T cells more completely including gamma-delta TcR T cells in all ALPS patients. In addition, expanded characterization of the B cells in these patients has also been initiated.

Funding Agency

• Agency: National Institute of Health (NIH)
• Institute: Clinical Center (CLC)
• Type: Intramural Research (Z01)
• Project #: 1Z01CL010287-03
• Application #: 6289482
• Study Section: Cognition and Perception Study Section (CP)
• Support Year: 3
• Fiscal Year: 1999

Institution

• Name: Clinical Center
• Country: United States

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