Table 1

Characteristics of patients and LGL expansions

Patient 1Patient 2Patient 3Patient 4Patient 5Patient 6Patient 7Patient 8
Characteristics of patients
Sex/age (years)F/55M/62F/82F/80F/80F/52F/67F/67
pSS disease duration (years)712519142
Subjective drynessYesYesYesYesYesYesYesYes
Schirmer test (mm)3315NA04NA0
USF (mL/min)*0.08
Sialadenitis: focus score3.41.61.71NA2.62.103.2
Past or present pSS systemic manifestationsArthralgia, Interstitial nephritis, Lymphoma (remission at LGLe diagnosis)No (in particular, no signs or symptoms of RA)Auto-immune thrombopeniaArthralgiaPolyarthritis, Peripheral neuropathy, MyositisArthralgia, ILD, glomerulonephritis, CryoglobulinemiaArthralgia, Pulmonary AL amyloidosis, CryoglobulinemiaPeripheral neuropathy, Cryoglobulinemia
(highest ever/at LGLe diagnosis)
Treatment received for pSSHCQ
RTX and Bendamustin (for lymphoma)
Characteristics of LGLe
Total lymphocytes count (/mm3)8483081243072508908503300960
LGL on blood smearNoNoYesNoNoNoNoNo
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%)
ClonalityT CD8+: oligoclonalT CD8+: oligoclonalNANAT CD8+: oligoclonal
T CD4: polyclonal
T CD8+: monoclonal
T CD4+: oligoclonal
T CD8+: monoclonalT CD8+: oligoclonal
Time from first RTX injection to LGLe diagnosis4 months15 days7 months7 months12 months
Neutrophil count (/mm3):
Before RTX2680NRNRNR3800210017505000
At LGLe diagnosis22410047032004340870980700
Nadir/time from first RTX injection (years)10/4 months68/NR470/NR2850/NR3370/NR870/7 months980/7months700/12 months
InfectionsRecurrent sinusitisNoNoNoPneumocystosisNoNoNo
Treatment of neutropeniaMTX, G-CSFMTX, G-CSFNoneNoneNoneNoneNoneNone
Therapeutic response, outcomeRemission of neutropenia after 3 monthsImprovement of neutropenia (500–1000/mm3) after 2 monthsPersistant neutropenia (500–1000/mm3)Remission of neutropenia after 2 monthsPersistant 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.