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Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency)‏

Published byStéphanie Blanchette Modified over 5 years ago

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Presentation on theme: "Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency)‏"— Presentation transcript:

1 Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency)‏ by Jana Pachlopnik Schmid, Danielle Canioni, Despina Moshous, Fabien Touzot, Nizar Mahlaoui, Fabian Hauck, Hirokazu Kanegane, Eduardo Lopez-Granados, Ester Mejstrikova, Isabelle Pellier, Lionel Galicier, Claire Galambrun, Vincent Barlogis, Pierre Bordigoni, Alain Fourmaintraux, Mohamed Hamidou, Alain Dabadie, Françoise Le Deist, Filomeen Haerynck, Marie Ouachée-Chardin, Pierre Rohrlich, Jean-Louis Stephan, Christelle Lenoir, Stéphanie Rigaud, Nathalie Lambert, Michèle Milili, Claudin Schiff, Helen Chapel, Capucine Picard, Geneviève de Saint Basile, Stéphane Blanche, Alain Fischer, and Sylvain Latour Blood Volume 117(5): February 3, 2011 ©2011 by American Society of Hematology

2 Comparison of HLH phenotypes and survival curves of SAP-deficient (XLP-1) and XIAP-deficient (XLP-2) patients. Comparison of HLH phenotypes and survival curves of SAP-deficient (XLP-1) and XIAP-deficient (XLP-2) patients. Kaplan-Meier survival curves were constructed on the basis of data presented in Table 1 and Table 2. Statistical analyses with log-rank tests. (A) Percentage of XLP-1/SAP and XLP-2/XIAP patients without HLH phenotype (P = .099). (B) Overall survival curves for XLP-1/SAP and XLP-2/XIAP patients (P = .948). Jana Pachlopnik Schmid et al. Blood 2011;117: ©2011 by American Society of Hematology

3 Histology of the large bowel of PX1.7 with XIAP deficiency.
Histology of the large bowel of PX1.7 with XIAP deficiency. (Top left) On hematoxylin and eosin at low magnification (×100), a large ulceration is seen, indicated by an arrow. (Bottom left) Higher magnification (×200) shows a massive polymorphic inflammatory infiltrate associated with a crypt abscess (indicated by the arrow). (Central right) Immunostaining with anti-CD3 shows frequent lymphoid T cells (on the right, ×200), some of them express the activation marker CD25 (×400, inset). Jana Pachlopnik Schmid et al. Blood 2011;117: ©2011 by American Society of Hematology

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