Characteristics of patients and LGL expansions
| Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | Patient 6 | Patient 7 | Patient 8 | |
| Characteristics of patients | ||||||||
| Sex/age (years) | F/55 | M/62 | F/82 | F/80 | F/80 | F/52 | F/67 | F/67 |
| pSS disease duration (years) | 7 | 1 | 2 | 5 | 1 | 9 | 14 | 2 |
| Subjective dryness | Yes | Yes | Yes | Yes | Yes | Yes | Yes | Yes |
| Schirmer test (mm) | 3 | 3 | 15 | NA | 0 | 4 | NA | 0 |
| USF (mL/min) | 0.3 | 0.2 | 0.2 | NA | 0.15 | 0.2 | NA* | 0.08 |
| Sialadenitis: focus score | 3.4 | 1.6 | 1.71 | NA | 2.6 | 2.1 | 0 | 3.2 |
| Past or present pSS systemic manifestations | Arthralgia, Interstitial nephritis, Lymphoma (remission at LGLe diagnosis) | No (in particular, no signs or symptoms of RA) | Auto-immune thrombopenia | Arthralgia | Polyarthritis, Peripheral neuropathy, Myositis | Arthralgia, ILD, glomerulonephritis, Cryoglobulinemia | Arthralgia, Pulmonary AL amyloidosis, Cryoglobulinemia | Peripheral neuropathy, Cryoglobulinemia |
| Anti-SSA/anti-SSB | Yes/No | Yes/Yes | No/No | Yes/No | No/No | No/No | No/No | Yes/Yes |
| Anti-CCP/RF | No/Yes | Yes/Yes | No/No | No/No | No/Yes | No/Yes | No/Yes | No/Yes |
| ESSDAI (highest ever/at LGLe diagnosis) | 52/6 | 20/14 | 8/8 | 3/3 | 35/35 | 51/11 | 10/10 | 22/22 |
| Treatment received for pSS | HCQ RTX and Bendamustin (for lymphoma) | None | None | None | Prednisone MTX, RTX | Prednisone RTX | RTX | RTX |
| Characteristics of LGLe | ||||||||
| Total lymphocytes count (/mm3) | 848 | 3081 | 2430 | 7250 | 890 | 850 | 3300 | 960 |
| LGL on blood smear | No | No | Yes | No | No | No | No | No |
| LGL type (% of total CD8+, CD4+ or NK cells) | T CD8+ (72%) and CD4+ (21%) | T CD8+ (38%) | LGL T CD8+ (% of CD8 cells not available) | NK (95%) | T CD8+ (61%) and CD4+ (20%) | T CD8+ (69%) and CD4+ (26%) | T CD8+ (37%) | T CD8+ (60%) |
| LGL cell count, /mm3 (% of total lymphocyte count) | 288 (34%) | 627 (20%) | 440 (18%) | 6000 (86%) | 180 (20%) | 750 (64%) | 1770 (57%) | 163 (17%) |
| Clonality | T CD8+: oligoclonal | T CD8+: oligoclonal | NA | NA | T CD8+: oligoclonal T CD4: polyclonal | T CD8+: monoclonal T CD4+: oligoclonal | T CD8+: monoclonal | T CD8+: oligoclonal |
| STAT3 mutation | No | No | NA | NA | NA | NA | NA | No |
| Time from first RTX injection to LGLe diagnosis | 4 months | 15 days | 7 months | 7 months | 12 months | |||
| Neutrophil count (/mm3): | ||||||||
| Before RTX | 2680 | NR | NR | NR | 3800 | 2100 | 1750 | 5000 |
| At LGLe diagnosis | 224 | 100 | 470 | 3200 | 4340 | 870 | 980 | 700 |
| Nadir/time from first RTX injection (years) | 10/4 months | 68/NR | 470/NR | 2850/NR | 3370/NR | 870/7 months | 980/7months | 700/12 months |
| Infections | Recurrent sinusitis | No | No | No | Pneumocystosis | No | No | No |
| Treatment of neutropenia | MTX, G-CSF | MTX, G-CSF | None | None | None | None | None | None |
| Therapeutic response, outcome | Remission of neutropenia after 3 months | Improvement of neutropenia (500–1000/mm3) after 2 months | Persistant neutropenia (500–1000/mm3) | Remission of neutropenia after 2 months | Persistant neutropenia (1000–1500/mm3) | Persistant neutropenia (500–1000/mm3) | ||
*Salivary gland scintigraphy showed a decrease in secretory function of parotid and submandibular gland.
ESSDAI, EULAR Sjögren’s syndrome disease activity index ; F, female ; HCQ, hydroxychloroquine ; ILD, interstitial lung disease ;LGL, large granular lymphocyte; M, male ; MTX, methotrexate ; NA, not available ;NR, not relevant; pSS, primary Sjögren’s syndrome ; RA, rheumatoid arthritis ; RF, rheumatoid factor ; RTX, rituximab; USF, unstimulated salivary flow.