Table 2.
Expression profile for each immunophenotypic marker investigated on mast cells from normal/reactive BM vs. systemic mastocytosis patients distributed according to the diagnostic/prognostic category of the disease.
| Normal BM (N = 40) |
SM (N = 166) |
WDSM (N = 18) |
ISMMC (N = 70) |
ISMML (N = 41) |
ISM-AHN (N = 12) |
ASM (N = 10) |
ASM-AHN (N = 9) |
MCL (N = 6) |
||
|---|---|---|---|---|---|---|---|---|---|---|
| CD22 | N | 9 | 57 | 8 | 10 | 19 | 6 | 4 | 6 | 4 |
| % positive patients | 78 | 77 | 75 | 90 | 79 | 83 | 75 | 83 | 25 | |
| p-value | - | - | - | ¥ p = 0.02 |
¥ p = 0.03 |
- | - | - | §$ p ≤ 0.03 |
|
| % positive cells | 89 (0–100) |
93 (0–100) |
100 (100–100) |
100 (100–100) |
100 (100–100) |
83 (0–100) |
75 (0–100) |
100 (100–100) |
50 (0–100) |
|
| p-value | - | - | ¥ p = 0.03 |
¥ p = 0.02 |
¶¥ p = 0.03 |
- | $ p = 0.03 |
- | †§$ p ≤ 0.03 |
|
| MFI | 1333 (8–3431) |
2629 (1–11,398) |
2187 (192–6614) |
2363 (424–5081) |
2959 (348–11,398) |
3086 (293–8612) |
3521 (246–6760) |
2841 (455–4435) |
721 (1–2398) |
|
| p-value | - | - | - | - | ¥ p = 0.006 |
- | - | ¥ p = 0.04 |
$‡ p ≤ 0.04 |
|
| CD25 | N | 40 | 166 | 18 | 70 | 41 | 12 | 10 | 9 | 6 |
| % positive patients | 0 | 89 | 33 | 99 | 100 | 92 | 100 | 100 | 33 | |
| p-value | - | * p = 0.001 |
§$‖¶‡ p ≤ 0.002 |
†¥ p = 0.001 |
†¥ p = 0.001 |
†¥ p ≤ 0.009 |
†¥ p ≤ 0.003 |
†¥ p ≤ 0.004 |
§$‖¶‡ p ≤ 0.009 |
|
| % positive cells | 0 (0–0) |
92 (0–100) |
44 (0–100) |
100 (100–100) |
100 (100–100) |
100 (100–100) |
100 (100–100) |
100 (100–100) |
33 (0–100) |
|
| p-value | - | * p = 0.001 |
§$‖¶‡ p ≤ 0.005 |
†¥ p = 0.001 |
†¥ p = 0.001 |
†¥ p ≤ 0.002 |
†¥ p = 0.003 |
†¥ p ≤ 0.005 |
§$‖¶‡ p ≤ 0.004 |
|
| MFI | 0 (0–0) |
5331 (4–21,943) |
471 (4–4159) |
6448 (806–17,552) |
5427 (2016–14,639) |
6544 (2329–16,474) |
6862 (1406–21,943) |
5371 (2376–11,108) |
1190 (21–4922) |
|
| p-value | - | * p = 0.001 |
§$‖¶‡ p = 0.001 |
†¥ p = 0.001 |
†¥ p = 0.001 |
†¥ p = 0.001 |
†¥ p ≤ 0.005 |
†¥ p ≤ 0.008 |
§$‖¶‡ p ≤ 0.008 |
|
| CD30 | N | 37 | 164 | 18 | 69 | 41 | 12 | 10 | 9 | 5 |
| % positive patients | 5 | 63 | 61 | 61 | 76 | 58 | 70 | 67 | 0 | |
| p-value | - | * p = 0.001 |
¥ p = 0.02 |
¥ p = 0.008 |
¥ p = 0.001 |
¥ p = 0.03 |
¥ p = 0.01 |
¥ p = 0.02 |
†§$‖¶‡ p ≤ 0.03 |
|
| % positive cells | 0 (0–0) |
88 (0–100) |
94 (0–100) |
87 (0–100) |
90 (0–100) |
83 (0–100) |
90 (0–100) |
89 (0–100) |
0 (0–0) |
|
| p-value | - | * p = 0.001 |
¥ p = 0.001 |
¥ p = 0.001 |
¥ p = 0.001 |
¥ p = 0.001 |
¥ p = 0.001 |
¥ p = 0.001 |
†§$‖¶‡ p = 0.001 |
|
| MFI | 129 (1–427) |
887 (0–6261) |
632 (202–1677) |
860 (0–3040) |
993 (199–3038) |
796 (103–2720) |
1095 (74–2847) |
1345 (202–6261) |
297 (153–495) |
|
| p-value | - | * p = 0.001 |
§$¥ p ≤ 0.02 |
†¥ p ≤ 0.02 |
†¥ p ≤ 0.01 |
- | - | ¥ p = 0.02 |
†§$‡ p ≤ 0.02 |
|
| CD33 | N | 31 | 114 | 11 | 60 | 22 | 7 | 7 | 4 | 3 |
| % positive patients | 100 | 100 | 100 | 100 | 100 | 100 | 100 | 100 | 100 | |
| p-value | - | - | - | - | - | - | - | - | - | |
| % positive cells | 100 (100–100) |
100 (100–100) |
100 (100–100) |
100 (100–100) |
100 (100–100) |
100 (100–100) |
100 (100–100) |
100 (100–100) |
100 (100–100) |
|
| p-value | - | - | - | - | - | - | - | - | - | |
| MFI | 8163 (2225–16,377) |
19,667 (2308–53,009) |
16,642 (5774–50,767) |
22,673 (4926–53,009) |
20,576 (2922–44,241) |
15,566 (5441–24,537) |
10,063 (2698–18,730) |
8447 (4022–14,916) |
10,917 (2308–23,681) |
|
| p-value | - | * p = 0.001 |
§ p = 0.03 |
†¶‡ p ≤ 0.03 |
¶ p = 0.02 |
- | §$ p ≤ 0.02 |
§ p = 0.002 |
- | |
| CD123 | N | 38 | 164 | 18 | 70 | 41 | 12 | 10 | 8 | 5 |
| % positive patients | 3 | 38 | 11 | 37 | 32 | 67 | 40 | 100 | 40 | |
| p-value | - | * p = 0.001 |
§‖‡ p ≤ 0.03 |
†‡ p ≤ 0.03 |
‖‡ p ≤ 0.03 |
†$ p ≤ 0.03 |
‡ p = 0.007 |
†§$¶¥ p ≤ 0.01 |
‡ p = 0.01 |
|
| % positive cells | 0 (0–0) |
64 (0–100) |
17 (0–100) |
66 (0–100) |
73 (0–100) |
70 (0–100) |
70 (0–100) |
100 (100–100) |
40 (0–100) |
|
| p-value | - | * p = 0.001 |
§$‖¶‡ p = 0.001 |
†‡ p ≤ 0.05 |
† p = 0.001 |
† p = 0.001 |
† p = 0.001 |
†§¥ p ≤ 0.05 |
‡ p = 0.01 |
|
| MFI | 84 (8–221) |
1340 (3–33 861) |
214 (3–2023) |
1024 (17–5425) |
962 (15–4045) |
3685 (24–33,861) |
939 (94–3220) |
5825 (755–18,421) |
903 (14–3085) |
|
| p-value | - | * p = 0.001 |
§$‖¶‡ p = 0.001 |
†‡ p = 0.001 |
†‡ p = 0.001 |
†‡ p ≤ 0.01 |
†‡ p ≤ 0.003 |
†§$‖¶¥ p ≤ 0.03 |
‡ p = 0.03 |
|
| FcεRI | N | 40 | 161 | 18 | 69 | 37 | 12 | 10 | 9 | 6 |
| % positive patients | 100 | 92 | 94 | 99 | 89 | 100 | 60 | 89 | 67 | |
| p-value | - | - | ¶ p = 0.02 |
$¶¥ p ≤ 0.03 |
§¶ p = 0.03 |
¶¥ p ≤ 0.03 |
†§$‖ p ≤ 0.03 |
- | §‖ p ≤ 0.03 |
|
| % positive cells | 100 (100–100) |
95 (0–100) |
94 (0–100) |
100 (100–100) |
95 (0–100) |
100 (100–100) |
70 (0–100) |
100 (100–100) |
67 (0–100) |
|
| p-value | - | - | § p = 0.05 |
†¶¥ p ≤ 0.05 |
¶¥ p = 0.001 |
¶¥ p = 0.04 |
§$‖ p ≤ 0.04 |
- | §$‖ p ≤ 0.03 |
|
| MFI | 29,073 (2181–81,199) |
49,387 (14–239,335) |
40,350 (110–183,969) |
78,922 (1271–239,335) |
24,084 (73–162,320) |
62,685 (1938–177,643) |
4858 (176–26,255) |
11,084 (680–36,688) |
9909 (14–52,514) |
|
| p-value | - | - | §¶¥ p ≤ 0.02 |
†$¶‡¥ p ≤ 0.002 |
§‖¶ p ≤ 0.04 |
$¶‡¥ p ≤ 0.04 |
†§$‖ p ≤ 0.005 |
§‖ p ≤ 0.04 |
†§‖ p ≤ 0.02 |
Results expressed as median values and range between brackets. Abbreviations: BM, bone marrow, WDSM, well-differentiated SM, ISMMC, indolent SM with KIT mutation restricted to mast cells, ISMML, ISM with multi-lineal KIT mutation, ISM-AHN, ISM associated with a clonal non-mast cell lineage hematopoietic disease, ASM, aggressive SM, ASM-AHN, ASM associated with a clonal non-mast cell lineage hematopoietic disease, MCL, mast cell leukemia, SI, stain index is the difference in signal width between the positive and the negative population divided by the signal spread of the negative population, and MFI, median fluorescence intensity (arbitrary values scaled from 0 to 222 144). p-values < 0.05 were considered to be associated with statistical significance for comparisons against: * normal/reactive BM, † WDSM, § ISMMC, $ ISMML, ‖ ISM-AHN, ¶ ASM, ‡ ASM-AHN, and ¥ MCL cases.