Table 3.
Clinical and biological features of clonal T/NK-LGLL patients (n = 82) distributed according to their STAT3 or STAT5B mutational status.
| Clinical and Biological Features | T/NK-LGLL | ||
|---|---|---|---|
| STAT3 and STAT5B Wild-Type (n = 54) |
STAT3 or STAT5B Mutated (n = 28) |
p-Value | |
| Sex (male/female) * | 23/31 (43%/57%) | 13/15 (46%/54%) | NS |
| Age (years) | 60 ± 18 (4–92) | 65 ± 15 (40–90) | NS |
| Physical examination | |||
| Organomegalies *1 | 5/34 (15%) | 9/27 (33%) | NS (0.09) |
| Skin lesions * | 4/33 (12%) 2 | 0/24 (0%) | NS |
| Peripheral blood cell counts | |||
| Hemoglobin (g/dL) | 13 ± 2.1 (8.3–18) | 13 ± 2.4 (6.3–17) | NS (0.08) |
| Platelets (×109/L) | 243 ± 72 (98–383) | 203 ± 88 (25–421) | 0.05 |
| Leukocytes (×109/L) | 10 ± 8 (2.7–55) | 6.7 ± 6.7 (0.9–36) | NS (0.07) |
| Clonal LGL cells (×109/L) | 4.1 ± 8.9 (0.07–50) | 4.1 ± 6.7 (0.5–30) | NS |
| Low-count clonal LGL lymphocytosis (<0.5 × 109/L) * | 14/47 (30%) | 6/28 (21%) | NS |
| Very low-count clonal LGL lymphocytosis (<0.1 × 109/L) * | 5/47 (11%) | 2/28 (7%) | NS |
| Cytopenias | |||
| Anemia (≤10 g/dL) * | 5/46 (11%) | 5/27 (19%) | NS |
| Thrombocytopenia (≤100 × 109/L) * | 1/45 (2%) | 3/27 (11%) | NS |
| Neutropenia (≤1 × 109/L) * | 6/47 (13%) | 9/28 (32%) | 0.04 |
| Severe Neutropenia (≤0.5 × 109/L) * | 0/47 (0%) | 4/28 (14%) | 0.02 |
| Other associated diseases | |||
| Other clonal/neoplastic diseases * | 10/32 (31%) | 4/25 (16%) | NS |
| Autoimmune diseases * (including cytopenias) |
16/35 (46%) | 18/28 (64%) | NS |
| Autoimmune diseases * (other than cytopenias) |
5/33 (15%) | 7/28 (25%) | NS |
| Other diseases * | 10/33 (30%) | 10/23 (43%) | NS |
| Outcome and follow-up | |||
| Need for LGLL therapy *3 | 4/44 (9%) | 12/24 (50%) | 0.0001 |
| Time to LGLL therapy (months) # | Not reached $ | 72 (1–180) | 0.0001 |
| Disease Progression * | 2/36 (6%) | 3/22 (14%) | NS |
| Deaths * (overall deaths) | 8/44 (18%) | 4/24 (17%) | NS |
| Deaths *4 | 1/44 (2%) | 2/23 (9%) | NS |
Results expressed as mean ± standard deviation (SD) (and range), * as number of cases (percentage) or # as median (95% confidence interval). In bold: statistically significant differences (p-value ≤ 0.05). $ After a median follow at of 183. 1 Adenopathy, splenomegaly, and/or hepatomegaly. 2 Cutaneous lesions corresponding to skin lesions of a coexisting mastocytosis, scleroderma, oro-buccal lesions, and cutaneous follicular NHL. 3 In all cases, treatment was administered because of the presence of cytopenias and/or other associated autoimmune diseases: WT cases were treated with cyclophosphamide (n = 2), corticoids (n = 1), and corticoids plus methotrexate (n = 1); STAT3 mutated cases were treated with cyclophosphamide (n = 1), methotrexate (n = 2), corticoids (n = 2), cyclosporine A (n = 2), and combinations of any of the above therapeutic agents plus corticoids and/or hematopoietic colony stimulating factors (n = 5). 4 Complications derived from the LGLL-associated autoimmune disease. Abbreviations (alphabetical order): LGLL, large granular lymphocytic leukemia; NS, no statistically significant differences (p-value > 0.05).