Clinical and biological features of 47 NK-LGLL patients and the diminished/ normal CD56 groups
Characteristic . | NK-LGLL (N = 47) . | Diminished CD56 group . | Normal CD56 group . | P value (diminished CD56 group vs normal CD56 group) . |
---|---|---|---|---|
(N = 28) . | (N = 19) . | |||
Median follow-up [min; max]-month | 56.2 [6.7;112.3] | 45.8 [6.7; 112.3] | 56.4 [12.8; 107.2] | .678 |
Age at diagnosis-years [min; max] | 52 [23; 82] | 53 [25; 82] | 52 [23; 70] | .522 |
Gender Male | 31/47 (66.0%) | 25/28 (89.3%) | 6/19 (31.6%) | <.001 |
Median LGL [min; max]- × 109/L | 3.7 [0.04;16.9] | 0.9 [0.04; 10.4] | 7.6 [0.2; 16.9] | <.001 |
Need for treatment | 27/47 (57.4%) | 24/28 (85.7%) | 3/19 (15.8%) | <.001 |
Symptoms at first visit | 29/47 (61.7%) | 26/28 (92.9%) | 3/9 (15.8%) | <.001 |
HB <120 g/L | 27/47 (57.4%) | 24/28 (85.7%) | 3/19 (15.8%) | <.001 |
HB <90 g/L | 18/47 (38.3%) | 18/28 (64.3%) | 0/19 (0.0%) | <.001 |
ANC <1.5 × 109/L | 19/47 (40.4%) | 19/28 (67.9%) | 0/19 (0.0%) | <.001 |
ANC <0.5 × 109/L | 2/47 (4.3%) | 2/28 (7.1%) | 0/19 (0.0%) | .350 |
PLTs <100 × 109/L | 6/47 (12.8%) | 5/28 (17.9%) | 1/19 (5.3%) | .410 |
Splenomegaly | 14/47 (29.8%) | 12/28 (42.9%) | 2/19 (10.5%) | .024 |
ECOG-PS | .105 | |||
0 | 34/47 (72.3%) | 17/28 (60.7%) | 17/19 (89.5%) | |
1 | 10/47 (21.7%) | 9/28 (32.1%) | 1/19 (5.3%) | |
2 | 3/47 (6.5%) | 2/28 (7.1%) | 1/19 (5.3%) | |
Autoimmune diseases | 7/47 (14.9%) | 6/28 (21.4%) | 1/19 (5.3%) | .267 |
PRCA | 6/43 (14.0%) | 6/26 (23.1%) | 0/17 (0.0%) | .092 |
Secondary primary malignancies | 2/47 (4.3%) | 0/28 (0.0%) | 2/19 (10.5%) | .158 |
LDH increased | 13/42 (31.0%) | 11/24 (45.8%) | 2/18 (11.1%) | .016 |
CD2 expression | 41/41 (100.0%) | |||
CD3 expression | 0/47 (0.0%) | |||
CD8 expression | 11/45 (24.5%) | 4/24 (14.3%) | 7/17 (41.2%) | .093 |
CD16 expression | 37/47 (78.7%) | 24/28 (85.7%) | 13/19 (68.4%) | .290 |
CD56 expression | ||||
Positive | 22/47 (46.8%) | |||
Partial positive | 8/47 (17.0%) | |||
Negative | 17/47 (36.2%) | |||
CD56 MIF | ||||
Bright or moderate | 19/22 (86.4%) | |||
Dim | 3/22 (13.6%) | |||
CD57 expression | 27/43 (62.8%) | 14/27 (51.9%) | 13/16 (81.3%) | .101 |
CD94 expression | 30/38 (78.9%) | 14/21 (66.7%) | 16/17 (94.1%) | .096 |
CD161 expression | 12/38 (31.6%) | 12/22 (54.5%) | 0/16 (0.0%) | <.001 |
Perforin | 35/41 (85.4%) | 22/25 (88.0%) | 13/16 (81.3%) | .886 |
Granzyme B | 33/41 (80.5%) | 21/25 (84.0%) | 12/16 (75.0%) | .760 |
KIR expression | .832 | |||
CD158a/b/e/f/i negative | 23/37 (62.2%) | 14/22 (63.6%) | 9/15 (60.0%) | |
Restrictive expression | 14/37 (37.8%) | 8/22 (36.4%) | 6/15 (40.0%) | |
CD158i | 10/37 (27.0%) | 7/22 (31.8%) | 3/15 (20.0%) | |
CD158a | 3/37 (8.1%) | 2/22 (9.1%) | 1/15 (6.7%) | |
CD158b | 2/37 (5.4%) | 0/22 (0.0%) | 2/15(13.3%) | |
STAT3 mutation | 13/32 (40.6%) | 13/21 (61.9%) | 0/11 (0.0%) | .003 |
STAT5B mutation | 0/32 (0.0%) | |||
LGL in bone marrow [min; max] % | 21.1 [2.4; 51.1] | 17.3 [2.4; 47.2] | 22.8 [6.2; 51.1] | .135 |
Bone marrow fibrosis | .006 | |||
0 | 10/42 (23.8%) | 2/26 (7.7%) | 8/16 (50.0%) | |
1 | 30/42 (71.4%) | 22/26 (84.6%) | 8/16 (50.0%) | |
2 | 2/42 (4.8%) | 2/26 (7.7%) | 0/16 (0.0%) |
Characteristic . | NK-LGLL (N = 47) . | Diminished CD56 group . | Normal CD56 group . | P value (diminished CD56 group vs normal CD56 group) . |
---|---|---|---|---|
(N = 28) . | (N = 19) . | |||
Median follow-up [min; max]-month | 56.2 [6.7;112.3] | 45.8 [6.7; 112.3] | 56.4 [12.8; 107.2] | .678 |
Age at diagnosis-years [min; max] | 52 [23; 82] | 53 [25; 82] | 52 [23; 70] | .522 |
Gender Male | 31/47 (66.0%) | 25/28 (89.3%) | 6/19 (31.6%) | <.001 |
Median LGL [min; max]- × 109/L | 3.7 [0.04;16.9] | 0.9 [0.04; 10.4] | 7.6 [0.2; 16.9] | <.001 |
Need for treatment | 27/47 (57.4%) | 24/28 (85.7%) | 3/19 (15.8%) | <.001 |
Symptoms at first visit | 29/47 (61.7%) | 26/28 (92.9%) | 3/9 (15.8%) | <.001 |
HB <120 g/L | 27/47 (57.4%) | 24/28 (85.7%) | 3/19 (15.8%) | <.001 |
HB <90 g/L | 18/47 (38.3%) | 18/28 (64.3%) | 0/19 (0.0%) | <.001 |
ANC <1.5 × 109/L | 19/47 (40.4%) | 19/28 (67.9%) | 0/19 (0.0%) | <.001 |
ANC <0.5 × 109/L | 2/47 (4.3%) | 2/28 (7.1%) | 0/19 (0.0%) | .350 |
PLTs <100 × 109/L | 6/47 (12.8%) | 5/28 (17.9%) | 1/19 (5.3%) | .410 |
Splenomegaly | 14/47 (29.8%) | 12/28 (42.9%) | 2/19 (10.5%) | .024 |
ECOG-PS | .105 | |||
0 | 34/47 (72.3%) | 17/28 (60.7%) | 17/19 (89.5%) | |
1 | 10/47 (21.7%) | 9/28 (32.1%) | 1/19 (5.3%) | |
2 | 3/47 (6.5%) | 2/28 (7.1%) | 1/19 (5.3%) | |
Autoimmune diseases | 7/47 (14.9%) | 6/28 (21.4%) | 1/19 (5.3%) | .267 |
PRCA | 6/43 (14.0%) | 6/26 (23.1%) | 0/17 (0.0%) | .092 |
Secondary primary malignancies | 2/47 (4.3%) | 0/28 (0.0%) | 2/19 (10.5%) | .158 |
LDH increased | 13/42 (31.0%) | 11/24 (45.8%) | 2/18 (11.1%) | .016 |
CD2 expression | 41/41 (100.0%) | |||
CD3 expression | 0/47 (0.0%) | |||
CD8 expression | 11/45 (24.5%) | 4/24 (14.3%) | 7/17 (41.2%) | .093 |
CD16 expression | 37/47 (78.7%) | 24/28 (85.7%) | 13/19 (68.4%) | .290 |
CD56 expression | ||||
Positive | 22/47 (46.8%) | |||
Partial positive | 8/47 (17.0%) | |||
Negative | 17/47 (36.2%) | |||
CD56 MIF | ||||
Bright or moderate | 19/22 (86.4%) | |||
Dim | 3/22 (13.6%) | |||
CD57 expression | 27/43 (62.8%) | 14/27 (51.9%) | 13/16 (81.3%) | .101 |
CD94 expression | 30/38 (78.9%) | 14/21 (66.7%) | 16/17 (94.1%) | .096 |
CD161 expression | 12/38 (31.6%) | 12/22 (54.5%) | 0/16 (0.0%) | <.001 |
Perforin | 35/41 (85.4%) | 22/25 (88.0%) | 13/16 (81.3%) | .886 |
Granzyme B | 33/41 (80.5%) | 21/25 (84.0%) | 12/16 (75.0%) | .760 |
KIR expression | .832 | |||
CD158a/b/e/f/i negative | 23/37 (62.2%) | 14/22 (63.6%) | 9/15 (60.0%) | |
Restrictive expression | 14/37 (37.8%) | 8/22 (36.4%) | 6/15 (40.0%) | |
CD158i | 10/37 (27.0%) | 7/22 (31.8%) | 3/15 (20.0%) | |
CD158a | 3/37 (8.1%) | 2/22 (9.1%) | 1/15 (6.7%) | |
CD158b | 2/37 (5.4%) | 0/22 (0.0%) | 2/15(13.3%) | |
STAT3 mutation | 13/32 (40.6%) | 13/21 (61.9%) | 0/11 (0.0%) | .003 |
STAT5B mutation | 0/32 (0.0%) | |||
LGL in bone marrow [min; max] % | 21.1 [2.4; 51.1] | 17.3 [2.4; 47.2] | 22.8 [6.2; 51.1] | .135 |
Bone marrow fibrosis | .006 | |||
0 | 10/42 (23.8%) | 2/26 (7.7%) | 8/16 (50.0%) | |
1 | 30/42 (71.4%) | 22/26 (84.6%) | 8/16 (50.0%) | |
2 | 2/42 (4.8%) | 2/26 (7.7%) | 0/16 (0.0%) |
Abbreviations: ANC, absolute neutrophil count; ECOG-PS, Eastern Cooperative Oncology Group performance status; HB, hemoglobin;KIR, Ig-like NK receptors; LGL, large granular lymphocyte; PLT, platelet; PRCA, pure red blood cell aplastic anemia.
Clinical and biological features of 47 NK-LGLL patients and the diminished/ normal CD56 groups
Characteristic . | NK-LGLL (N = 47) . | Diminished CD56 group . | Normal CD56 group . | P value (diminished CD56 group vs normal CD56 group) . |
---|---|---|---|---|
(N = 28) . | (N = 19) . | |||
Median follow-up [min; max]-month | 56.2 [6.7;112.3] | 45.8 [6.7; 112.3] | 56.4 [12.8; 107.2] | .678 |
Age at diagnosis-years [min; max] | 52 [23; 82] | 53 [25; 82] | 52 [23; 70] | .522 |
Gender Male | 31/47 (66.0%) | 25/28 (89.3%) | 6/19 (31.6%) | <.001 |
Median LGL [min; max]- × 109/L | 3.7 [0.04;16.9] | 0.9 [0.04; 10.4] | 7.6 [0.2; 16.9] | <.001 |
Need for treatment | 27/47 (57.4%) | 24/28 (85.7%) | 3/19 (15.8%) | <.001 |
Symptoms at first visit | 29/47 (61.7%) | 26/28 (92.9%) | 3/9 (15.8%) | <.001 |
HB <120 g/L | 27/47 (57.4%) | 24/28 (85.7%) | 3/19 (15.8%) | <.001 |
HB <90 g/L | 18/47 (38.3%) | 18/28 (64.3%) | 0/19 (0.0%) | <.001 |
ANC <1.5 × 109/L | 19/47 (40.4%) | 19/28 (67.9%) | 0/19 (0.0%) | <.001 |
ANC <0.5 × 109/L | 2/47 (4.3%) | 2/28 (7.1%) | 0/19 (0.0%) | .350 |
PLTs <100 × 109/L | 6/47 (12.8%) | 5/28 (17.9%) | 1/19 (5.3%) | .410 |
Splenomegaly | 14/47 (29.8%) | 12/28 (42.9%) | 2/19 (10.5%) | .024 |
ECOG-PS | .105 | |||
0 | 34/47 (72.3%) | 17/28 (60.7%) | 17/19 (89.5%) | |
1 | 10/47 (21.7%) | 9/28 (32.1%) | 1/19 (5.3%) | |
2 | 3/47 (6.5%) | 2/28 (7.1%) | 1/19 (5.3%) | |
Autoimmune diseases | 7/47 (14.9%) | 6/28 (21.4%) | 1/19 (5.3%) | .267 |
PRCA | 6/43 (14.0%) | 6/26 (23.1%) | 0/17 (0.0%) | .092 |
Secondary primary malignancies | 2/47 (4.3%) | 0/28 (0.0%) | 2/19 (10.5%) | .158 |
LDH increased | 13/42 (31.0%) | 11/24 (45.8%) | 2/18 (11.1%) | .016 |
CD2 expression | 41/41 (100.0%) | |||
CD3 expression | 0/47 (0.0%) | |||
CD8 expression | 11/45 (24.5%) | 4/24 (14.3%) | 7/17 (41.2%) | .093 |
CD16 expression | 37/47 (78.7%) | 24/28 (85.7%) | 13/19 (68.4%) | .290 |
CD56 expression | ||||
Positive | 22/47 (46.8%) | |||
Partial positive | 8/47 (17.0%) | |||
Negative | 17/47 (36.2%) | |||
CD56 MIF | ||||
Bright or moderate | 19/22 (86.4%) | |||
Dim | 3/22 (13.6%) | |||
CD57 expression | 27/43 (62.8%) | 14/27 (51.9%) | 13/16 (81.3%) | .101 |
CD94 expression | 30/38 (78.9%) | 14/21 (66.7%) | 16/17 (94.1%) | .096 |
CD161 expression | 12/38 (31.6%) | 12/22 (54.5%) | 0/16 (0.0%) | <.001 |
Perforin | 35/41 (85.4%) | 22/25 (88.0%) | 13/16 (81.3%) | .886 |
Granzyme B | 33/41 (80.5%) | 21/25 (84.0%) | 12/16 (75.0%) | .760 |
KIR expression | .832 | |||
CD158a/b/e/f/i negative | 23/37 (62.2%) | 14/22 (63.6%) | 9/15 (60.0%) | |
Restrictive expression | 14/37 (37.8%) | 8/22 (36.4%) | 6/15 (40.0%) | |
CD158i | 10/37 (27.0%) | 7/22 (31.8%) | 3/15 (20.0%) | |
CD158a | 3/37 (8.1%) | 2/22 (9.1%) | 1/15 (6.7%) | |
CD158b | 2/37 (5.4%) | 0/22 (0.0%) | 2/15(13.3%) | |
STAT3 mutation | 13/32 (40.6%) | 13/21 (61.9%) | 0/11 (0.0%) | .003 |
STAT5B mutation | 0/32 (0.0%) | |||
LGL in bone marrow [min; max] % | 21.1 [2.4; 51.1] | 17.3 [2.4; 47.2] | 22.8 [6.2; 51.1] | .135 |
Bone marrow fibrosis | .006 | |||
0 | 10/42 (23.8%) | 2/26 (7.7%) | 8/16 (50.0%) | |
1 | 30/42 (71.4%) | 22/26 (84.6%) | 8/16 (50.0%) | |
2 | 2/42 (4.8%) | 2/26 (7.7%) | 0/16 (0.0%) |
Characteristic . | NK-LGLL (N = 47) . | Diminished CD56 group . | Normal CD56 group . | P value (diminished CD56 group vs normal CD56 group) . |
---|---|---|---|---|
(N = 28) . | (N = 19) . | |||
Median follow-up [min; max]-month | 56.2 [6.7;112.3] | 45.8 [6.7; 112.3] | 56.4 [12.8; 107.2] | .678 |
Age at diagnosis-years [min; max] | 52 [23; 82] | 53 [25; 82] | 52 [23; 70] | .522 |
Gender Male | 31/47 (66.0%) | 25/28 (89.3%) | 6/19 (31.6%) | <.001 |
Median LGL [min; max]- × 109/L | 3.7 [0.04;16.9] | 0.9 [0.04; 10.4] | 7.6 [0.2; 16.9] | <.001 |
Need for treatment | 27/47 (57.4%) | 24/28 (85.7%) | 3/19 (15.8%) | <.001 |
Symptoms at first visit | 29/47 (61.7%) | 26/28 (92.9%) | 3/9 (15.8%) | <.001 |
HB <120 g/L | 27/47 (57.4%) | 24/28 (85.7%) | 3/19 (15.8%) | <.001 |
HB <90 g/L | 18/47 (38.3%) | 18/28 (64.3%) | 0/19 (0.0%) | <.001 |
ANC <1.5 × 109/L | 19/47 (40.4%) | 19/28 (67.9%) | 0/19 (0.0%) | <.001 |
ANC <0.5 × 109/L | 2/47 (4.3%) | 2/28 (7.1%) | 0/19 (0.0%) | .350 |
PLTs <100 × 109/L | 6/47 (12.8%) | 5/28 (17.9%) | 1/19 (5.3%) | .410 |
Splenomegaly | 14/47 (29.8%) | 12/28 (42.9%) | 2/19 (10.5%) | .024 |
ECOG-PS | .105 | |||
0 | 34/47 (72.3%) | 17/28 (60.7%) | 17/19 (89.5%) | |
1 | 10/47 (21.7%) | 9/28 (32.1%) | 1/19 (5.3%) | |
2 | 3/47 (6.5%) | 2/28 (7.1%) | 1/19 (5.3%) | |
Autoimmune diseases | 7/47 (14.9%) | 6/28 (21.4%) | 1/19 (5.3%) | .267 |
PRCA | 6/43 (14.0%) | 6/26 (23.1%) | 0/17 (0.0%) | .092 |
Secondary primary malignancies | 2/47 (4.3%) | 0/28 (0.0%) | 2/19 (10.5%) | .158 |
LDH increased | 13/42 (31.0%) | 11/24 (45.8%) | 2/18 (11.1%) | .016 |
CD2 expression | 41/41 (100.0%) | |||
CD3 expression | 0/47 (0.0%) | |||
CD8 expression | 11/45 (24.5%) | 4/24 (14.3%) | 7/17 (41.2%) | .093 |
CD16 expression | 37/47 (78.7%) | 24/28 (85.7%) | 13/19 (68.4%) | .290 |
CD56 expression | ||||
Positive | 22/47 (46.8%) | |||
Partial positive | 8/47 (17.0%) | |||
Negative | 17/47 (36.2%) | |||
CD56 MIF | ||||
Bright or moderate | 19/22 (86.4%) | |||
Dim | 3/22 (13.6%) | |||
CD57 expression | 27/43 (62.8%) | 14/27 (51.9%) | 13/16 (81.3%) | .101 |
CD94 expression | 30/38 (78.9%) | 14/21 (66.7%) | 16/17 (94.1%) | .096 |
CD161 expression | 12/38 (31.6%) | 12/22 (54.5%) | 0/16 (0.0%) | <.001 |
Perforin | 35/41 (85.4%) | 22/25 (88.0%) | 13/16 (81.3%) | .886 |
Granzyme B | 33/41 (80.5%) | 21/25 (84.0%) | 12/16 (75.0%) | .760 |
KIR expression | .832 | |||
CD158a/b/e/f/i negative | 23/37 (62.2%) | 14/22 (63.6%) | 9/15 (60.0%) | |
Restrictive expression | 14/37 (37.8%) | 8/22 (36.4%) | 6/15 (40.0%) | |
CD158i | 10/37 (27.0%) | 7/22 (31.8%) | 3/15 (20.0%) | |
CD158a | 3/37 (8.1%) | 2/22 (9.1%) | 1/15 (6.7%) | |
CD158b | 2/37 (5.4%) | 0/22 (0.0%) | 2/15(13.3%) | |
STAT3 mutation | 13/32 (40.6%) | 13/21 (61.9%) | 0/11 (0.0%) | .003 |
STAT5B mutation | 0/32 (0.0%) | |||
LGL in bone marrow [min; max] % | 21.1 [2.4; 51.1] | 17.3 [2.4; 47.2] | 22.8 [6.2; 51.1] | .135 |
Bone marrow fibrosis | .006 | |||
0 | 10/42 (23.8%) | 2/26 (7.7%) | 8/16 (50.0%) | |
1 | 30/42 (71.4%) | 22/26 (84.6%) | 8/16 (50.0%) | |
2 | 2/42 (4.8%) | 2/26 (7.7%) | 0/16 (0.0%) |
Abbreviations: ANC, absolute neutrophil count; ECOG-PS, Eastern Cooperative Oncology Group performance status; HB, hemoglobin;KIR, Ig-like NK receptors; LGL, large granular lymphocyte; PLT, platelet; PRCA, pure red blood cell aplastic anemia.
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