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When patients presenting HLH are presumed to have XLP, we recommend flow cytometric analysis or Western blot of SAP and XIAP expression followed by gene analysis. Allogeneic HSCT with RIC regime is recommended in patients with SAP deficiency. The pathogenesis of XLP, especially XIAP deficiency, is not fully understood, and extensive studies are required in the future. This study was supported by Grants-in-Aid for Scientific Research from the Ministry of Education, Culture, Sports, Science and Technology, and grants from the Ministry of Health, Labour, and Welfare of Japan. We thank Dr Sylvain Latour for critical discussion, and are grateful to many doctors for providing us with samples and patient data. ""63239" "Anti-platelet therapy for Kawasaki Ozagrel disease (KD) is often done without monitoring drug Apoptosis inhibitor efficacy. The aim of this study was to investigate the utility of whole-blood aggregometry to evaluate the efficacy of anti-platelet therapy for KD. Of 37 late-phase KD patients included in the present study, 20 were prescribed anti-platelet drugs. Platelet-rich plasma (PRP) aggregation with collagen as the stimulus was measured using an optical aggregometer. The area under the curve of small and large size aggregations was calculated, and categorized into five classes: �C2, �C1, 0, 1, and 2. Whole-blood aggregation with collagen or adenosine 5��-diphosphate (ADP) as stimulus was evaluated using the platelet aggregation threshold index (PATI), which is the concentration of stimulus that induces a whole-blood aggregation rate of 50%. In both collagen- and ADP-induced aggregation, there was a negative correlation between PATI and class determination using the PRP technique (collagen, rs = ?0.870, P Galunisertib cell line Moreover, the PATI in collagen- and ADP-induced aggregation was significantly higher in the anti-platelet drug therapy group than in the untreated group (collagen, P