Clinical and biologic presentation: data from the largest retrospective series
| Parameters . | Loughran et al (1993)115 . | Semenzato et al (1997)80 . | Bareau et al (2010)2 . | Sanikommu et al (2017)70 . | Zhu et al (2020)116 . | Dong et al (2021)71 . |
|---|---|---|---|---|---|---|
| Number of patients | 129 | 162 | 229 | 204 | 108 | 319 |
| T-LGLL (%) | 100 | 100 | 88 | 90 | 100 | 93 |
| NK-LGLL (%) | NA (aggressive form) | 0 | 12 | 10 | 0 | 8 |
| Median age, y (min-max) | 57 (15-88) | 59 | 59 (12-87) | 63 (54-72) | 51 (21-78) | 65 (17-90) |
| Sex ratio (M/F) | 0.8 | 0.8 | 0.8 | 0.9 | 0.9 | 0.9 |
| Biological features | ||||||
| LGL count (×109/L) | NA | NA | <1 × 109/L, 37% >4 × 109/L, 15% | 1.7 (0.8-3.3) | 2.07 (0.3-45.5) | 0.9 (0.5-2.3) |
| Median neutrophil count (min-max) | NA | NA | NA | 1.56 (0.8-2.6) | 1.36 (0.04-9.6) | NA |
| Neutropenia (%) (ANC <1.5 × 109/L) | 84 | NA | 59 | 46 | 57 | 41 |
| Severe neutropenia (ANC <0.5 × 109/L) (%) | 48 | 37 | 24 | 17 | 10 | 17 |
| Median Hb level (min-max) | NA | NA | NA | 11.7 (10-13) | 7.5 (3.1-15.6) | NA |
| Anemia (%) | 49 | 26 | 24 | 40 | 59 | 41 |
| Transfusion dependency (%) | NA | NA | 6 | 22 | 58 | 19 |
| Thrombocytopenia (%) | 19 | 9 | 17 | 30 | 8 | 26 |
| Antinuclear antibodies | 38 | 38 | 17 | NA | 45 | 22 |
| Rheumatoid factor (%) | 57 | 43 | 14 | NA | 10 | 39 |
| STAT3 mutation (%) | NA | NA | NA | 36 | 28 | 40∗ |
| Clinical manifestations | ||||||
| Treatment requirement | 73 | 33 | 44 | 58 | 97 | 57 |
| B symptoms (%) | NA | 26 | 7 | NA | 0 | 63 |
| Hepatomegaly (%) | 23 | 32 | 10 | NA | 6 | NA |
| Splenomegaly (%) | 50 | 44 | 24 | 24 | 41 | 28 |
| Lymphadenopathies (%) | 1 | 12 | 6 | NA | 7 | NA |
| Recurrent infections (%) | 39 | 61 | 22 | NA | 12 | NA |
| Autoimmune manifestations (%) | 28 | NA | 32 | 25 | 23 | 26 |
| RA (%) | 28 | NA | 17 | 15 | 2.7 | 12 |
| Other hematological malignancies (%) | NA | NA | 10 | 19 | 0 | 19 |
| Solid tumor (%) | NA | NA | 4 | 17 | 0 | 21 |
| LGL-related death (%) | 36 | NA | 7 | 5 | 3 | NA |
| Parameters . | Loughran et al (1993)115 . | Semenzato et al (1997)80 . | Bareau et al (2010)2 . | Sanikommu et al (2017)70 . | Zhu et al (2020)116 . | Dong et al (2021)71 . |
|---|---|---|---|---|---|---|
| Number of patients | 129 | 162 | 229 | 204 | 108 | 319 |
| T-LGLL (%) | 100 | 100 | 88 | 90 | 100 | 93 |
| NK-LGLL (%) | NA (aggressive form) | 0 | 12 | 10 | 0 | 8 |
| Median age, y (min-max) | 57 (15-88) | 59 | 59 (12-87) | 63 (54-72) | 51 (21-78) | 65 (17-90) |
| Sex ratio (M/F) | 0.8 | 0.8 | 0.8 | 0.9 | 0.9 | 0.9 |
| Biological features | ||||||
| LGL count (×109/L) | NA | NA | <1 × 109/L, 37% >4 × 109/L, 15% | 1.7 (0.8-3.3) | 2.07 (0.3-45.5) | 0.9 (0.5-2.3) |
| Median neutrophil count (min-max) | NA | NA | NA | 1.56 (0.8-2.6) | 1.36 (0.04-9.6) | NA |
| Neutropenia (%) (ANC <1.5 × 109/L) | 84 | NA | 59 | 46 | 57 | 41 |
| Severe neutropenia (ANC <0.5 × 109/L) (%) | 48 | 37 | 24 | 17 | 10 | 17 |
| Median Hb level (min-max) | NA | NA | NA | 11.7 (10-13) | 7.5 (3.1-15.6) | NA |
| Anemia (%) | 49 | 26 | 24 | 40 | 59 | 41 |
| Transfusion dependency (%) | NA | NA | 6 | 22 | 58 | 19 |
| Thrombocytopenia (%) | 19 | 9 | 17 | 30 | 8 | 26 |
| Antinuclear antibodies | 38 | 38 | 17 | NA | 45 | 22 |
| Rheumatoid factor (%) | 57 | 43 | 14 | NA | 10 | 39 |
| STAT3 mutation (%) | NA | NA | NA | 36 | 28 | 40∗ |
| Clinical manifestations | ||||||
| Treatment requirement | 73 | 33 | 44 | 58 | 97 | 57 |
| B symptoms (%) | NA | 26 | 7 | NA | 0 | 63 |
| Hepatomegaly (%) | 23 | 32 | 10 | NA | 6 | NA |
| Splenomegaly (%) | 50 | 44 | 24 | 24 | 41 | 28 |
| Lymphadenopathies (%) | 1 | 12 | 6 | NA | 7 | NA |
| Recurrent infections (%) | 39 | 61 | 22 | NA | 12 | NA |
| Autoimmune manifestations (%) | 28 | NA | 32 | 25 | 23 | 26 |
| RA (%) | 28 | NA | 17 | 15 | 2.7 | 12 |
| Other hematological malignancies (%) | NA | NA | 10 | 19 | 0 | 19 |
| Solid tumor (%) | NA | NA | 4 | 17 | 0 | 21 |
| LGL-related death (%) | 36 | NA | 7 | 5 | 3 | NA |
ANC, absolute neutrophils count; F, female; Hb, hemoglobin; M, male; max, maximum; min, minimum; NA, not available.
Performed only on 25 patients.