Source

Sex  

Age at diagnosis

Clinical mani-festations

Lymphocyte count at diag-nosis (*10^9/L)

Clonality

Immuno-phenotypic markers

Laboratory findings

Associated auto-immune or hema-tological disease

Treatment under-gone for SS and LGL

Molad et al. 2001

F

65

Arthralgia, keratoc-onjunctivitis sicca, fatigue, weight loss

2.9 (maximum value reached: 4.5)

Not determined

CD8+, CD57+, CD16+

RF +, ANA +, anti-Ro antibodies +

None reported

Cyclosporin A

Molad et al. 2001

F

70

Neutropenia, xero-pthlamia, xerostom-ia, fatigue, arthritis

6.3

Not determined

CD8+, CD57+/-

RF +, ANA +

Hashimoto thyroiditis

Not reported

Ergas et al. 2002

F

30

Pure red cell aplasia, amegak-aryocytic throm-bo-cytopenic pu-rpura, xeropthal-mia, xerostomia

2.5

Clonal re-arrangement of the TCR gene

CD8+, CD45R+

None reported

Hashimoto tyroiditis, premature ovarian failure

Azathioprine, blood and platelet transfu-sions, high dose in-travenous immuneo-globulin, cyclospori-n A, methotrexate, cyclophosphamide

Friedman et al. 2006

F

44

Pure red cell aplasia, agranulocytosis, immmune thrombo-cytopenia

7.8

 Clonal rearrangement of the TCR gene in all but one patient

CD8+

None reported

None reported

Not reported

Friedman et al. 2006

F

68

None reported

4.2

CD4+, CD57+

Antithyroid

Polycystic ovarian syndrome, anemia of chronic disease

Not reported

antibodies +

Friedman et al. 2006

F

64

None reported

4.1

CD4+

Antithyroid

Angioneurotic edema

Not reported

antibodies +

Friedman et al. 2006

F

72

None reported

2.8

CD8+, CD57+

None reported

Diabetes, anemia of chronic disease

Not reported

Friedman et al. 2006

F

73

None reported

3.8

CD8+, CD56+

None reported

CREST syndrome

Not reported

Friedman et al. 2006

F

75

None reported

3.1

CD4+

None reported

Hashimoto thyroiditis

Not reported

Friedman et al. 2006

F

66

Agranulocytosis, immune thrombocy-topenia

4.3

CD8+

None reported

Hyperparathyroidism

Not reported

Friedman et al. 2006

F

64

Leukopenia

1

CD8+

None reported

Hashimoto thyroiditis, vitiligo

Not reported

Friedman et al. 2006

F

61

Agranulocytosis

1.2

CD8+

None reported

Diabetes

Not reported

Friedman et al. 2006

F

43

Agranulocytosis,  Raynaud's phenomenon

0.8

CD8+

Monoclonal IgG k component

None reported

Not reported

Friedman et al. 2006

F

66

None reported

2.3

CD4+

Antithyroid

None reported

Not reported

antibodies +

Friedman et al. 2006

F

63

None reported

9.6

CD8+, CD57+

None reported

Hashimoto thyroiditis

Not reported

Friedman et al. 2006

F

71

None reported

7.3

CD8+, CD56+

None reported

None reported

Not reported

Franco et al. 2010

F

73

Neutropenia, fever, splenomegaly, cerv-ical lymphadenopat-hy, xerofthalmia, xerostomia

1.7

Clonal rear-angement of the TCR gene

CD8+, CD57+, CD56-

Polyclonal hyp-ergammaglobulinemia, monocl-onal IgA lamb-da component,

Chronic disease related anemia

Methotrexate

RF +, ANA +

Baber et al. 2019

F

55

Arthralgia, interstitial nephritis, neutropenia

8.5

Oligoclonal TCR beta chain rear-rangement

CD8+

Anti-SSA antib-odies and RF +

Lymphoma

Hydroxy-chloroqui-ne, R-bendamustine, methotrexate, G-CSF

Baber et al. 2019

M

62

Neutropenia

3.1

Oligoclonal TCR beta chain rear-rangement

CD8+

Anti-SSA and anti-SSB antib-odies and RF +

None reported

None

Baber et al. 2019

F

82

Autoimmune thrombo-cytopenia, neutropenia

2.4

Not determined

CD8+

None reported

None reported

None

Baber et al. 2019

F

80

Arthralgia, neutropenia

7.3

Not determined

NK cells

Anti-SSA antibodies +

None reported

None

Baber et al. 2019

F

80

Polyartritis, periphe-ral neuropathy, my-ositis, neutropenia

0.9

Oligoclonal TCR beta chain rearran-gement

CD8+

RF +

None reported

Prednisone, methotrexate, rituximab

Baber et al. 2019

F

52

Arthralgia, interstitial lung disease, glomerulo-nephritis, cryo-globulinemia, neutropenia

0.9

TCR beta cha-in rearrange-ent monoclon-al in CD8+ T cells and oli-goclonal in CD4+ T cells

CD8+

RF +

None reported

Prednisone, rituximab

Baber et al. 2019

F

67

Arthralgia, cryo-globulinemia, neutropenia

3.3

Monoclonal TCR beta chain rearra-ngement

CD8+

RF +

Pulmonary AL amyloidosis

Rituximab

Baber et al. 2019

F

67

Peripheral neuropathy, cryglobulinemia, neutropenia

1

Oligoclonal TCR beta chain rear-angement

CD8+, CD57+/-, CD45RA+

Anti-SSA and anti-SSB antibodies and RF +

None reported

Rituximab

Tavarozzi et al. 2020

F

39

Xerophtalmia, xerostomia, anemia, thrombo-cytopenia

8.2

Monoclonal TCR beta chain rear-angement

CD8+, CD57+/-, CD45RA+

ANA, ENA, and anti-SSA antibodies +

None reported

Hydroxy-chloroquine