Table 1 The clinical and laboratory characteristics of patients with CLPD-NK in previous studies [2,3,4,5,6,7,8,9,10,11,12,13,14]
Country/[reference] | Year | Patient number | Sex (M: F) | Age (median) | Neutropenia | Anaemia | Thrombocytopenia | Symptomatic | Fatigue | B symptoms | Hepatomegaly | Splenomegaly | Lymphadenopathy | Infection | EBV | STAT3 mutation |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
USA/[4] | 1994 | 10 | 6: 4 | 60 | 3 | 2 | 2 | 7 | NA | 1 | 0 | 1 | 0 | 1 | NA | NA |
USA/ [5] | 1999 | 16 | 14: 2 | 60.5 (7–78) | 5 | 2 | 1 | 9 | 0 | 4 | 0 | 1 | 0 | NA | NA | NA |
Spain/ [6] | 2004 | 26 | 11: 15 | 68 ± 17 (37–94) | 11 | 12 | 7 | 3 | NA | NA | 1 | 4 | 3 | 1 | NA | NA |
Japan/ [7] | 2005 | 19 | 10: 9 | 55 (14–69) | NA | NA | NA | NA | NA | NA | 1 | 2 | 0 | NA | 6/7 | NA |
USA/ [8] | 2012 | 50 | 28: 22 | 61 (27–90) | 30 | 15 | 8 | 27 | NA | NA | NA | 6 | NA | NA | NA | 15/50 |
China/ [9] | 2013 | 17 | 10: 7 | 65 (45–81) | NA | 10 | NA | NA | NA | 3 | 1 | 1 | 1 | NA | 1/5 | NA |
China/ [10] | 2014 | 8 | 2: 6 | 55.5 (28–75) | 5 | 1 | 1 | 4 | 1 | 0 | 0 | 1 | 1 | 1 | NA | NA |
France/ [3] | 2014 | 70 | 41: 29 | 61 | 23 | 13 | 14 | 36 | 18 | 10 | 9 | 10 | 4 | 10 | NA | 5/40 |
Italia/ [11] | 2014 | 48 | 34: 14 | 61 | 13 | 4 | 3 | 23 | NA | NA | NA | 14 | NA | NA | NA | 4/48 |
China/ [12] | 2016 | 9 | 5: 4 | 37 (16–62) | NA | NA | NA | NA | NA | NA | 5 | 2 | 1 | NA | 5/9 | NA |
China/ [13] | 2016 | 13 | 8: 5 | 54 (51–86) | 7 | 7 | 2 | 9 | 4 | 4 | 0 | 0 | 4 | 0 | 1/13 | NA |
Italia/ [14] | 2018 | 25 | NA | 62 (42–79) | 10 | 2 | 3 | NA | NA | NA | NA | NA | NA | NA | NA | NA |
USA/ [2] | 2018 | 11 | 7: 4 | 60 (25–89) | 9 | 9 | 6 | 10 | 6 | 4 | 0 | 4 | 4 | 5 | 0/1 | 7/10 |
Total |  | 322 | 176: 121 |  | 116/277 (41.9 %) | 77/294 (26.2 %) | 47/277 (17.0 %) | 128/252 (50.8 %) | 29/118 (24.6 %) | 26/145 (17.9 %) | 17/199 (8.5 %) | 46/297 (15.5 %) | 18/199 (9.0 %) | 18/138 (13.0 %) | 13/35 (37.1 %) | 31/148 (20.9 %) |