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Natural history of autoimmune lymphoproliferative syndrome associated with FAS gene mutations by Susan Price, Pamela A. Shaw, Amy Seitz, Gyan Joshi, Joie.

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Presentation on theme: "Natural history of autoimmune lymphoproliferative syndrome associated with FAS gene mutations by Susan Price, Pamela A. Shaw, Amy Seitz, Gyan Joshi, Joie."— Presentation transcript:

1 Natural history of autoimmune lymphoproliferative syndrome associated with FAS gene mutations by Susan Price, Pamela A. Shaw, Amy Seitz, Gyan Joshi, Joie Davis, Julie E. Niemela, Katie Perkins, Ronald L. Hornung, Les Folio, Philip S. Rosenberg, Jennifer M. Puck, Amy P. Hsu, Bernice Lo, Stefania Pittaluga, Elaine S. Jaffe, Thomas A. Fleisher, V. Koneti Rao, and Michael J. Lenardo Blood Volume 123(13):1989-1999 March 27, 2014 ©2014 by American Society of Hematology

2 Signal transduction by the FAS receptor whose gene is mutated in ALPS as detailed in the Introduction. Susan Price et al. Blood 2014;123:1989-1999 ©2014 by American Society of Hematology

3 Kaplan-Meier curve for lymphoma risk in ALPS-FAS (N = 150). Susan Price et al. Blood 2014;123:1989-1999 ©2014 by American Society of Hematology

4 Splenectomy is a common intervention that leads to sepsis but does not prevent cytopenias. Susan Price et al. Blood 2014;123:1989-1999 ©2014 by American Society of Hematology

5 Causes and consequences of splenectomy in ALPS-FAS. Susan Price et al. Blood 2014;123:1989-1999 ©2014 by American Society of Hematology

6 Kaplan-Meier curve for overall survival (N = 150). Susan Price et al. Blood 2014;123:1989-1999 ©2014 by American Society of Hematology


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