Table 1.

Clinical and biological features of 47 NK-LGLL patients and the diminished/ normal CD56 groups

CharacteristicNK-LGLL (N = 47)Diminished CD56 groupNormal CD56 groupP value (diminished CD56 group vs normal CD56 group)
(N = 28)(N = 19)
Median follow-up [min; max]-month56.2 [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 Male31/47 (66.0%)25/28 (89.3%)6/19 (31.6%)<.001
Median LGL [min; max]- × 109/L3.7 [0.04;16.9]0.9 [0.04; 10.4]7.6 [0.2; 16.9]<.001
Need for treatment27/47 (57.4%)24/28 (85.7%)3/19 (15.8%)<.001
Symptoms at first visit29/47 (61.7%)26/28 (92.9%)3/9 (15.8%)<.001
HB <120 g/L27/47 (57.4%)24/28 (85.7%)3/19 (15.8%)<.001
HB <90 g/L18/47 (38.3%)18/28 (64.3%)0/19 (0.0%)<.001
ANC <1.5 × 109/L19/47 (40.4%)19/28 (67.9%)0/19 (0.0%)<.001
ANC <0.5 × 109/L2/47 (4.3%)2/28 (7.1%)0/19 (0.0%).350
PLTs <100 × 109/L6/47 (12.8%)5/28 (17.9%)1/19 (5.3%).410
Splenomegaly14/47 (29.8%)12/28 (42.9%)2/19 (10.5%).024
ECOG-PS.105
 034/47 (72.3%)17/28 (60.7%)17/19 (89.5%)
 110/47 (21.7%)9/28 (32.1%)1/19 (5.3%)
 23/47 (6.5%)2/28 (7.1%)1/19 (5.3%)
Autoimmune diseases7/47 (14.9%)6/28 (21.4%)1/19 (5.3%).267
PRCA6/43 (14.0%)6/26 (23.1%)0/17 (0.0%).092
Secondary primary malignancies2/47 (4.3%)0/28 (0.0%)2/19 (10.5%).158
LDH increased13/42 (31.0%)11/24 (45.8%)2/18 (11.1%).016
CD2 expression41/41 (100.0%)
CD3 expression0/47 (0.0%)
CD8 expression11/45 (24.5%)4/24 (14.3%)7/17 (41.2%).093
CD16 expression37/47 (78.7%)24/28 (85.7%)13/19 (68.4%).290
CD56 expression
Positive22/47 (46.8%)
Partial positive8/47 (17.0%)
Negative17/47 (36.2%)
CD56 MIF
Bright or moderate19/22 (86.4%)
Dim3/22 (13.6%)
CD57 expression27/43 (62.8%)14/27 (51.9%)13/16 (81.3%).101
CD94 expression30/38 (78.9%)14/21 (66.7%)16/17 (94.1%).096
CD161 expression12/38 (31.6%)12/22 (54.5%)0/16 (0.0%)<.001
Perforin35/41 (85.4%)22/25 (88.0%)13/16 (81.3%).886
Granzyme B33/41 (80.5%)21/25 (84.0%)12/16 (75.0%).760
KIR expression.832
 CD158a/b/e/f/i negative23/37 (62.2%)14/22 (63.6%)9/15 (60.0%)
Restrictive expression14/37 (37.8%)8/22 (36.4%)6/15 (40.0%)
  CD158i10/37 (27.0%)7/22 (31.8%)3/15 (20.0%)
  CD158a3/37 (8.1%)2/22 (9.1%)1/15 (6.7%)
  CD158b2/37 (5.4%)0/22 (0.0%)2/15(13.3%)
STAT3 mutation13/32 (40.6%)13/21 (61.9%)0/11 (0.0%).003
STAT5B mutation0/32 (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
 010/42 (23.8%)2/26 (7.7%)8/16 (50.0%)
 130/42 (71.4%)22/26 (84.6%)8/16 (50.0%)
 22/42 (4.8%)2/26 (7.7%)0/16 (0.0%)
CharacteristicNK-LGLL (N = 47)Diminished CD56 groupNormal CD56 groupP value (diminished CD56 group vs normal CD56 group)
(N = 28)(N = 19)
Median follow-up [min; max]-month56.2 [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 Male31/47 (66.0%)25/28 (89.3%)6/19 (31.6%)<.001
Median LGL [min; max]- × 109/L3.7 [0.04;16.9]0.9 [0.04; 10.4]7.6 [0.2; 16.9]<.001
Need for treatment27/47 (57.4%)24/28 (85.7%)3/19 (15.8%)<.001
Symptoms at first visit29/47 (61.7%)26/28 (92.9%)3/9 (15.8%)<.001
HB <120 g/L27/47 (57.4%)24/28 (85.7%)3/19 (15.8%)<.001
HB <90 g/L18/47 (38.3%)18/28 (64.3%)0/19 (0.0%)<.001
ANC <1.5 × 109/L19/47 (40.4%)19/28 (67.9%)0/19 (0.0%)<.001
ANC <0.5 × 109/L2/47 (4.3%)2/28 (7.1%)0/19 (0.0%).350
PLTs <100 × 109/L6/47 (12.8%)5/28 (17.9%)1/19 (5.3%).410
Splenomegaly14/47 (29.8%)12/28 (42.9%)2/19 (10.5%).024
ECOG-PS.105
 034/47 (72.3%)17/28 (60.7%)17/19 (89.5%)
 110/47 (21.7%)9/28 (32.1%)1/19 (5.3%)
 23/47 (6.5%)2/28 (7.1%)1/19 (5.3%)
Autoimmune diseases7/47 (14.9%)6/28 (21.4%)1/19 (5.3%).267
PRCA6/43 (14.0%)6/26 (23.1%)0/17 (0.0%).092
Secondary primary malignancies2/47 (4.3%)0/28 (0.0%)2/19 (10.5%).158
LDH increased13/42 (31.0%)11/24 (45.8%)2/18 (11.1%).016
CD2 expression41/41 (100.0%)
CD3 expression0/47 (0.0%)
CD8 expression11/45 (24.5%)4/24 (14.3%)7/17 (41.2%).093
CD16 expression37/47 (78.7%)24/28 (85.7%)13/19 (68.4%).290
CD56 expression
Positive22/47 (46.8%)
Partial positive8/47 (17.0%)
Negative17/47 (36.2%)
CD56 MIF
Bright or moderate19/22 (86.4%)
Dim3/22 (13.6%)
CD57 expression27/43 (62.8%)14/27 (51.9%)13/16 (81.3%).101
CD94 expression30/38 (78.9%)14/21 (66.7%)16/17 (94.1%).096
CD161 expression12/38 (31.6%)12/22 (54.5%)0/16 (0.0%)<.001
Perforin35/41 (85.4%)22/25 (88.0%)13/16 (81.3%).886
Granzyme B33/41 (80.5%)21/25 (84.0%)12/16 (75.0%).760
KIR expression.832
 CD158a/b/e/f/i negative23/37 (62.2%)14/22 (63.6%)9/15 (60.0%)
Restrictive expression14/37 (37.8%)8/22 (36.4%)6/15 (40.0%)
  CD158i10/37 (27.0%)7/22 (31.8%)3/15 (20.0%)
  CD158a3/37 (8.1%)2/22 (9.1%)1/15 (6.7%)
  CD158b2/37 (5.4%)0/22 (0.0%)2/15(13.3%)
STAT3 mutation13/32 (40.6%)13/21 (61.9%)0/11 (0.0%).003
STAT5B mutation0/32 (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
 010/42 (23.8%)2/26 (7.7%)8/16 (50.0%)
 130/42 (71.4%)22/26 (84.6%)8/16 (50.0%)
 22/42 (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.

Table 1.

Clinical and biological features of 47 NK-LGLL patients and the diminished/ normal CD56 groups

CharacteristicNK-LGLL (N = 47)Diminished CD56 groupNormal CD56 groupP value (diminished CD56 group vs normal CD56 group)
(N = 28)(N = 19)
Median follow-up [min; max]-month56.2 [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 Male31/47 (66.0%)25/28 (89.3%)6/19 (31.6%)<.001
Median LGL [min; max]- × 109/L3.7 [0.04;16.9]0.9 [0.04; 10.4]7.6 [0.2; 16.9]<.001
Need for treatment27/47 (57.4%)24/28 (85.7%)3/19 (15.8%)<.001
Symptoms at first visit29/47 (61.7%)26/28 (92.9%)3/9 (15.8%)<.001
HB <120 g/L27/47 (57.4%)24/28 (85.7%)3/19 (15.8%)<.001
HB <90 g/L18/47 (38.3%)18/28 (64.3%)0/19 (0.0%)<.001
ANC <1.5 × 109/L19/47 (40.4%)19/28 (67.9%)0/19 (0.0%)<.001
ANC <0.5 × 109/L2/47 (4.3%)2/28 (7.1%)0/19 (0.0%).350
PLTs <100 × 109/L6/47 (12.8%)5/28 (17.9%)1/19 (5.3%).410
Splenomegaly14/47 (29.8%)12/28 (42.9%)2/19 (10.5%).024
ECOG-PS.105
 034/47 (72.3%)17/28 (60.7%)17/19 (89.5%)
 110/47 (21.7%)9/28 (32.1%)1/19 (5.3%)
 23/47 (6.5%)2/28 (7.1%)1/19 (5.3%)
Autoimmune diseases7/47 (14.9%)6/28 (21.4%)1/19 (5.3%).267
PRCA6/43 (14.0%)6/26 (23.1%)0/17 (0.0%).092
Secondary primary malignancies2/47 (4.3%)0/28 (0.0%)2/19 (10.5%).158
LDH increased13/42 (31.0%)11/24 (45.8%)2/18 (11.1%).016
CD2 expression41/41 (100.0%)
CD3 expression0/47 (0.0%)
CD8 expression11/45 (24.5%)4/24 (14.3%)7/17 (41.2%).093
CD16 expression37/47 (78.7%)24/28 (85.7%)13/19 (68.4%).290
CD56 expression
Positive22/47 (46.8%)
Partial positive8/47 (17.0%)
Negative17/47 (36.2%)
CD56 MIF
Bright or moderate19/22 (86.4%)
Dim3/22 (13.6%)
CD57 expression27/43 (62.8%)14/27 (51.9%)13/16 (81.3%).101
CD94 expression30/38 (78.9%)14/21 (66.7%)16/17 (94.1%).096
CD161 expression12/38 (31.6%)12/22 (54.5%)0/16 (0.0%)<.001
Perforin35/41 (85.4%)22/25 (88.0%)13/16 (81.3%).886
Granzyme B33/41 (80.5%)21/25 (84.0%)12/16 (75.0%).760
KIR expression.832
 CD158a/b/e/f/i negative23/37 (62.2%)14/22 (63.6%)9/15 (60.0%)
Restrictive expression14/37 (37.8%)8/22 (36.4%)6/15 (40.0%)
  CD158i10/37 (27.0%)7/22 (31.8%)3/15 (20.0%)
  CD158a3/37 (8.1%)2/22 (9.1%)1/15 (6.7%)
  CD158b2/37 (5.4%)0/22 (0.0%)2/15(13.3%)
STAT3 mutation13/32 (40.6%)13/21 (61.9%)0/11 (0.0%).003
STAT5B mutation0/32 (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
 010/42 (23.8%)2/26 (7.7%)8/16 (50.0%)
 130/42 (71.4%)22/26 (84.6%)8/16 (50.0%)
 22/42 (4.8%)2/26 (7.7%)0/16 (0.0%)
CharacteristicNK-LGLL (N = 47)Diminished CD56 groupNormal CD56 groupP value (diminished CD56 group vs normal CD56 group)
(N = 28)(N = 19)
Median follow-up [min; max]-month56.2 [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 Male31/47 (66.0%)25/28 (89.3%)6/19 (31.6%)<.001
Median LGL [min; max]- × 109/L3.7 [0.04;16.9]0.9 [0.04; 10.4]7.6 [0.2; 16.9]<.001
Need for treatment27/47 (57.4%)24/28 (85.7%)3/19 (15.8%)<.001
Symptoms at first visit29/47 (61.7%)26/28 (92.9%)3/9 (15.8%)<.001
HB <120 g/L27/47 (57.4%)24/28 (85.7%)3/19 (15.8%)<.001
HB <90 g/L18/47 (38.3%)18/28 (64.3%)0/19 (0.0%)<.001
ANC <1.5 × 109/L19/47 (40.4%)19/28 (67.9%)0/19 (0.0%)<.001
ANC <0.5 × 109/L2/47 (4.3%)2/28 (7.1%)0/19 (0.0%).350
PLTs <100 × 109/L6/47 (12.8%)5/28 (17.9%)1/19 (5.3%).410
Splenomegaly14/47 (29.8%)12/28 (42.9%)2/19 (10.5%).024
ECOG-PS.105
 034/47 (72.3%)17/28 (60.7%)17/19 (89.5%)
 110/47 (21.7%)9/28 (32.1%)1/19 (5.3%)
 23/47 (6.5%)2/28 (7.1%)1/19 (5.3%)
Autoimmune diseases7/47 (14.9%)6/28 (21.4%)1/19 (5.3%).267
PRCA6/43 (14.0%)6/26 (23.1%)0/17 (0.0%).092
Secondary primary malignancies2/47 (4.3%)0/28 (0.0%)2/19 (10.5%).158
LDH increased13/42 (31.0%)11/24 (45.8%)2/18 (11.1%).016
CD2 expression41/41 (100.0%)
CD3 expression0/47 (0.0%)
CD8 expression11/45 (24.5%)4/24 (14.3%)7/17 (41.2%).093
CD16 expression37/47 (78.7%)24/28 (85.7%)13/19 (68.4%).290
CD56 expression
Positive22/47 (46.8%)
Partial positive8/47 (17.0%)
Negative17/47 (36.2%)
CD56 MIF
Bright or moderate19/22 (86.4%)
Dim3/22 (13.6%)
CD57 expression27/43 (62.8%)14/27 (51.9%)13/16 (81.3%).101
CD94 expression30/38 (78.9%)14/21 (66.7%)16/17 (94.1%).096
CD161 expression12/38 (31.6%)12/22 (54.5%)0/16 (0.0%)<.001
Perforin35/41 (85.4%)22/25 (88.0%)13/16 (81.3%).886
Granzyme B33/41 (80.5%)21/25 (84.0%)12/16 (75.0%).760
KIR expression.832
 CD158a/b/e/f/i negative23/37 (62.2%)14/22 (63.6%)9/15 (60.0%)
Restrictive expression14/37 (37.8%)8/22 (36.4%)6/15 (40.0%)
  CD158i10/37 (27.0%)7/22 (31.8%)3/15 (20.0%)
  CD158a3/37 (8.1%)2/22 (9.1%)1/15 (6.7%)
  CD158b2/37 (5.4%)0/22 (0.0%)2/15(13.3%)
STAT3 mutation13/32 (40.6%)13/21 (61.9%)0/11 (0.0%).003
STAT5B mutation0/32 (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
 010/42 (23.8%)2/26 (7.7%)8/16 (50.0%)
 130/42 (71.4%)22/26 (84.6%)8/16 (50.0%)
 22/42 (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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