Comparison between pAN, LO-Np, and LL-Np
| . | pAN (n = 135 patients) . | LO-Np (n =31 patients) . | LL-Np (n = 48 patients) . | P . |
|---|---|---|---|---|
| Female sex | 41/135 (30) | 16/31 (52) | 25/48 (52) | .001 |
| Age at diagnosis, median (IQR), y | 0.6 (0.3-1.3) | 11.5 (7.6-14.6) | 1.18 (0.6-2.2) | <.001 |
| Lenght of neutropenia, median (IQR), y | 1.03 (0.54-1.7) | 2.1 (1.4-4.4) | 4.5 (3.5-7.09) | <.001 |
| Resolution of neutropenia | 135/135 (100) | 4/31 (13) | 28/48(58) | <.001 |
| ANC at onset, median (IQR), ×109/L | 0.43 (0.23-0.716) | 0.649 (0.43-0.97) | 0.552 (0.35-0.79) | <.001 |
| Leukocyte count at onset, median (IQR), ×109/L | 6.125 (5.01-7.92) | 3.18 (2.67-3.71) | 5.03 (3.44-6.90) | <.001 |
| Lymphocyte count, median (IQR), ×109/L | 4.74 (3.50-5.88) | 1.68 (1.24-1.90) | 2.37 (1.92-3.40) | <.001 |
| Monocitosis at onset | 15/120 (12.5) | 5/26 (19) | 10/38 (26) | NS |
| Low CD3* | 7/72 (10) | 2/24 (8) | 11/39 (28) | .02 |
| Low CD4* | 8/72 (11) | 4/25 (16) | 11/38 (29) | .06 |
| Low CD8* | 11/71 (15) | 4/25 (16) | 7/40 (17,5) | NS |
| Low CD19* | 6/64 (9) | 12/25 (48) | 13/37 (35) | <.001 |
| Low NK* (CD3+CD16+CD56+) | 10/62 (16) | 9/23 (39) | 10/34 (29) | .06 |
| Immunoglobulin depletion* | 7/113 (6) | 4/26 (15) | 3/44 (7) | NS |
| Infectious episodes | 65/130 (50) | 14/29 (48) | 18/47 (38) | .4 |
| Severe infections | 16/65 (25) | 3/14(21) | 3/18 (17) | .2 |
| G-CSF therapy | 7/135 (5) | 3/21 (14) | 7/42 (17) | .04 |
| Autoimmune diseases/markers | 2/135 (1) | 16/29 (55) | 8/48 (17) | <.001 |
| . | pAN (n = 135 patients) . | LO-Np (n =31 patients) . | LL-Np (n = 48 patients) . | P . |
|---|---|---|---|---|
| Female sex | 41/135 (30) | 16/31 (52) | 25/48 (52) | .001 |
| Age at diagnosis, median (IQR), y | 0.6 (0.3-1.3) | 11.5 (7.6-14.6) | 1.18 (0.6-2.2) | <.001 |
| Lenght of neutropenia, median (IQR), y | 1.03 (0.54-1.7) | 2.1 (1.4-4.4) | 4.5 (3.5-7.09) | <.001 |
| Resolution of neutropenia | 135/135 (100) | 4/31 (13) | 28/48(58) | <.001 |
| ANC at onset, median (IQR), ×109/L | 0.43 (0.23-0.716) | 0.649 (0.43-0.97) | 0.552 (0.35-0.79) | <.001 |
| Leukocyte count at onset, median (IQR), ×109/L | 6.125 (5.01-7.92) | 3.18 (2.67-3.71) | 5.03 (3.44-6.90) | <.001 |
| Lymphocyte count, median (IQR), ×109/L | 4.74 (3.50-5.88) | 1.68 (1.24-1.90) | 2.37 (1.92-3.40) | <.001 |
| Monocitosis at onset | 15/120 (12.5) | 5/26 (19) | 10/38 (26) | NS |
| Low CD3* | 7/72 (10) | 2/24 (8) | 11/39 (28) | .02 |
| Low CD4* | 8/72 (11) | 4/25 (16) | 11/38 (29) | .06 |
| Low CD8* | 11/71 (15) | 4/25 (16) | 7/40 (17,5) | NS |
| Low CD19* | 6/64 (9) | 12/25 (48) | 13/37 (35) | <.001 |
| Low NK* (CD3+CD16+CD56+) | 10/62 (16) | 9/23 (39) | 10/34 (29) | .06 |
| Immunoglobulin depletion* | 7/113 (6) | 4/26 (15) | 3/44 (7) | NS |
| Infectious episodes | 65/130 (50) | 14/29 (48) | 18/47 (38) | .4 |
| Severe infections | 16/65 (25) | 3/14(21) | 3/18 (17) | .2 |
| G-CSF therapy | 7/135 (5) | 3/21 (14) | 7/42 (17) | .04 |
| Autoimmune diseases/markers | 2/135 (1) | 16/29 (55) | 8/48 (17) | <.001 |
Data are n/N (%) unless other noted. NS, not significant.
Values below the third percentile for age.