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) |
| 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)