Patient characteristics, clinical presentation, type of virus, biology at diagnosis, treatment, and outcomes
| . | Case series (n = 18) . | Literature review cases (n = 34) . | Pooled data (n = 52) . | Missing data . | |||
|---|---|---|---|---|---|---|---|
| No. (%) . | Median (range) . | No. (%) . | Median (range) . | No. (%) . | Median (range) . | No. (%) . | |
| Patient characteristics | |||||||
| Age, y | 4.4 (1.5-6) | 5.2 (1.8-11) | 4.9 (1.5-11) | — | |||
| Male sex | 7 (39) | 20 (59) | 27 (52) | — | |||
| Clinical presentation | |||||||
| Time to diagnosis, d | 6 (2-17) | 8 (5-15) | 7 (2-17) | — | |||
| Lower limb | 16 (89) | 33 (97) | 49 (94) | — | |||
| Calves only | 3 (17) | 10 (29) | 13 (25) | — | |||
| Extended leg | 13 (72) | 23 (68) | 36 (69) | — | |||
| Upper limb | 2 (11) | 5 (14) | 7 (13) | — | |||
| Genitalia | 1 (5.6) | 3 (9) | 4 (8) | — | |||
| Torso | 3 (17) | 8 (23) | 11 (21) | — | |||
| Virus | |||||||
| HHV-6 | 3 (17) | 1 (3) | 4 (8) | — | |||
| VZV | 11 (61) | 30 (86) | 41 (78) | — | |||
| Unknown | 4 (22) | 4 (11) | 7 (14) | — | |||
| Biology at diagnosis | |||||||
| PS activity, % | 5 (1-28) | 2.5 (1-25) | 4 (1-28) | 24 (52) | |||
| PS free antigen, % | 6 (1-16) | 1 (1-9) | 1 (1-16) | 24 (46) | |||
| PS total antigen, % | 4.5 (1-32) | 5.5 (1-62) | 5 (1-62) | 22 (42) | |||
| Anti-PS antibody | 13 (72) | 17 (50) | 30 (58) | — | |||
| PC activity, % | 43 (14-131) | 54 (14-100) | 49.5 (14-131) | 4 (8) | |||
| Antithrombin level, % | 85 (45-130) | 86 (48-115) | 85 (45-130) | 11 (21) | |||
| Fibrinogen level, g/L | 1.85 (0.35-3.4) | 0.5 (0.1-2.38) | 0.88 (0.1-3.4) | 10 (19) | |||
| Platelet count, ×109/L | 65 (10-250) | 166 (14-302) | 150 (10-302) | 8 (15) | |||
| Increase in D-dimer | 14 (78) | 16/18 (89) | 30/36 (83) | 17 (33) | |||
| Treatment | |||||||
| Heparin | 18 (100) | 33 (97) | 51 (98) | — | |||
| Corticosteroid | 3 (17) | 13 (37) | 16 (31) | — | |||
| Fresh frozen plasma | 13 (72) | 29 (83) | 41 (79) | — | |||
| Polyvalent Ig | 8 (44) | 11 (31) | 20 (38) | — | |||
| Plasmapheresis | 7 (39) | 6 (17) | 13 (25) | — | |||
| Coagulation regulator* | 5 (28) | 4 (11) | 12 (23) | — | |||
| Outcomes | |||||||
| Amputation | 6 (33) | 8 (24) | 14 (27) | — | |||
| Skin necrosis with graft | 5 (28) | 10 (29) | 15 (29) | — | |||
| Veinous thromboembolism | 6 (33) | 11 (32) | 17 (33) | — | |||
| Hemorrhagic complication | 2 (11) | ND | ND | — | |||
| . | Case series (n = 18) . | Literature review cases (n = 34) . | Pooled data (n = 52) . | Missing data . | |||
|---|---|---|---|---|---|---|---|
| No. (%) . | Median (range) . | No. (%) . | Median (range) . | No. (%) . | Median (range) . | No. (%) . | |
| Patient characteristics | |||||||
| Age, y | 4.4 (1.5-6) | 5.2 (1.8-11) | 4.9 (1.5-11) | — | |||
| Male sex | 7 (39) | 20 (59) | 27 (52) | — | |||
| Clinical presentation | |||||||
| Time to diagnosis, d | 6 (2-17) | 8 (5-15) | 7 (2-17) | — | |||
| Lower limb | 16 (89) | 33 (97) | 49 (94) | — | |||
| Calves only | 3 (17) | 10 (29) | 13 (25) | — | |||
| Extended leg | 13 (72) | 23 (68) | 36 (69) | — | |||
| Upper limb | 2 (11) | 5 (14) | 7 (13) | — | |||
| Genitalia | 1 (5.6) | 3 (9) | 4 (8) | — | |||
| Torso | 3 (17) | 8 (23) | 11 (21) | — | |||
| Virus | |||||||
| HHV-6 | 3 (17) | 1 (3) | 4 (8) | — | |||
| VZV | 11 (61) | 30 (86) | 41 (78) | — | |||
| Unknown | 4 (22) | 4 (11) | 7 (14) | — | |||
| Biology at diagnosis | |||||||
| PS activity, % | 5 (1-28) | 2.5 (1-25) | 4 (1-28) | 24 (52) | |||
| PS free antigen, % | 6 (1-16) | 1 (1-9) | 1 (1-16) | 24 (46) | |||
| PS total antigen, % | 4.5 (1-32) | 5.5 (1-62) | 5 (1-62) | 22 (42) | |||
| Anti-PS antibody | 13 (72) | 17 (50) | 30 (58) | — | |||
| PC activity, % | 43 (14-131) | 54 (14-100) | 49.5 (14-131) | 4 (8) | |||
| Antithrombin level, % | 85 (45-130) | 86 (48-115) | 85 (45-130) | 11 (21) | |||
| Fibrinogen level, g/L | 1.85 (0.35-3.4) | 0.5 (0.1-2.38) | 0.88 (0.1-3.4) | 10 (19) | |||
| Platelet count, ×109/L | 65 (10-250) | 166 (14-302) | 150 (10-302) | 8 (15) | |||
| Increase in D-dimer | 14 (78) | 16/18 (89) | 30/36 (83) | 17 (33) | |||
| Treatment | |||||||
| Heparin | 18 (100) | 33 (97) | 51 (98) | — | |||
| Corticosteroid | 3 (17) | 13 (37) | 16 (31) | — | |||
| Fresh frozen plasma | 13 (72) | 29 (83) | 41 (79) | — | |||
| Polyvalent Ig | 8 (44) | 11 (31) | 20 (38) | — | |||
| Plasmapheresis | 7 (39) | 6 (17) | 13 (25) | — | |||
| Coagulation regulator* | 5 (28) | 4 (11) | 12 (23) | — | |||
| Outcomes | |||||||
| Amputation | 6 (33) | 8 (24) | 14 (27) | — | |||
| Skin necrosis with graft | 5 (28) | 10 (29) | 15 (29) | — | |||
| Veinous thromboembolism | 6 (33) | 11 (32) | 17 (33) | — | |||
| Hemorrhagic complication | 2 (11) | ND | ND | — | |||
ND, not diagnosed.
Antithrombin and activated PC.