Table 1.

PDC numbers and MxA expression in WAS spleen

Case MxA score MxA pDCs Clinical score/autoimmunity 
  %   
WAS#1 4 18.52 6.21 5/+ 
WAS#2 2 13.23 203.92 5/− 
WAS#3 3 7.99 154.98 5/− 
WAS#4 1 2.89 228.76 5/+ 
WAS#5 4 19.02 50.40 3/− 
WAS#6 2 1.03 93.71 3/− 
WAS#7 4 13.95 323.48 5/+ 
WAS#8 4 13.61 8.68 5/+ 
Mean (±SD) 3 11.28 (±6.7) 133.8 (±113.9)  
CTR#1 3 14.44 131.15  
CTR#2 1 0.47 578.25  
CTR#3 2 1.6 202.63  
CTR#4 2 0.15 223.00  
CTR#5 2 4.21 NV  
CTR#6 4 25.29 202.63  
CTR#7 2 10.24 156.26  
CTR#8 2 0.61 62.95  
CTR#9 ne ne 83.21  
CTR#10 3 20.48 43.80  
Mean (±SD) 2.3 7.12 (±9.5) 187.10 (±160.30)  

ne = not evaluable. MxA is expressed as the percentage of reactivity of the spleen area. pDCs are expressed as BDCA-2+ cells/mm2spleen area. Absolute pDC count and MxA expression were quantified on spleen sections of WAS patients. The MxA score was evaluated by counting the percentage of MxA+ cells and expressed as a four-tier scale. MxA percentage of reactivity of the spleen area and BDCA-2+ cells/mm2 spleen area were evaluated automatically on digital slides as described in Materials and methods. Disease score is given according to the classification reported previously (Imai et al., 2004)

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