Clinical and hematologic features of patients with ES overall and by primary and secondary cases
| . | All patients (N = 116) . | Primary (n = 92) . | Secondary (n = 24) . |
|---|---|---|---|
| Demographics | |||
| Age, y | 50.2 (1.9-94.8) | 50.7 (1.9-94.8) | 52.6 (7.6-79.5) |
| Sex | |||
| Male | 57 (49) | 42 (46) | 15 (63) |
| Female | 59 (51) | 50 (54) | 9 (37) |
| Laboratory values | |||
| Hb, g/dL | 8.5 (2.8-17) | 8.9 (2.8-17.0) | 7.6 (3.7-13.5) |
| PLTs, × 109/L | 20 (1-727) | 20 (1-727) | 21 (1-324) |
| ANC, × 109/L | 3.0 (0.2-15.2) | 3.0 (0.2-14.3) | 2.8 (0.2-15.2) |
| LDH, ×ULN | 1.7 (0.5-16) | 1.7 (0.6-16) | 1.5 (0.5-11) |
| IgG, g/dL | 1002 (52-5660) | 1030 (52-5660) | 862 (113-1440) |
| IgA, g/dL | 155 (24-1257) | 145 (24-1257) | 143 (40-375) |
| IgM, g/dL | 88 (10-1800) | 83 (10-1800) | 88 (27-475) |
| DAT+ | 103 (89) | 80 (87) | 23 (96) |
| IgG | 64 (62) | 48 (60) | 16 (70) |
| IgG + C | 25 (24) | 20 (25) | 5 (22) |
| C | 5 (5) | 5 (6.25) | 0 (0) |
| Mixed | 7 (7) | 6 (7.5) | 1 (4) |
| Atypical | 2 (2) | 1 (1.25) | 1 (4) |
| DAT− AIHA* | 6 (5) | 5 (5) | 1 (4) |
| Positive anti-PLTs (n = 49) | 41 (84) | 37(82)† | 4 (100) |
| Positive antineutrophils (n = 42) | 10 (45) | 8 (42)‡ | 2 (67) |
| ANA (n = 94) | 34 (36) | 29/74 | 5/20 |
| ENA (n = 69) | 6 (7) | 4/57 | 2/12 |
| Anti-DNA (n = 72) | 4 (56) | 2/58 | 2/14 |
| Anticardiolipin antibodies (n = 70) | 12 (17) | 11/56 | 1/14 |
| Anti–β2 glycoprotein 1 antibodies (n = 51) | 9 (18) | 7/46 | 2/5 |
| LAC (n = 60) | 9 (15) | 4/49 | 5/11 |
| Disease categories | |||
| Cytopenia at onset | |||
| ITP | 39 (34) | 34 (37) | 5 (21) |
| AIHA | 36 (31) | 30 (33) | 6 (25) |
| AIN | 5 (4) | 4 (4) | 1 (4) |
| Concomitant | 36 (31) | 24 (26) | 12 (50) |
| ES type | |||
| ITP and AIHA | 94 (81) | 75 (82) | 19 (79) |
| ITP and AIN | 7 (6) | 6 (6) | 1 (4) |
| AIHA and AIN | 3 (3) | 3 (3) | 0 (0) |
| ITP, AIHA, and AIN | 12 (10) | 8 (9) | 4 (17) |
| Splenomegaly | 37 (32) | 26 (28) | 11 (46) |
| Complications and outcomes | |||
| Infection | 39 (34) | 26 (28) | 13 (54) |
| Thrombosis | 25 (22) | 17 (18) | 8 (33) |
| Death§ | 23 (21) | 19 (21) | 4 (17) |
| . | All patients (N = 116) . | Primary (n = 92) . | Secondary (n = 24) . |
|---|---|---|---|
| Demographics | |||
| Age, y | 50.2 (1.9-94.8) | 50.7 (1.9-94.8) | 52.6 (7.6-79.5) |
| Sex | |||
| Male | 57 (49) | 42 (46) | 15 (63) |
| Female | 59 (51) | 50 (54) | 9 (37) |
| Laboratory values | |||
| Hb, g/dL | 8.5 (2.8-17) | 8.9 (2.8-17.0) | 7.6 (3.7-13.5) |
| PLTs, × 109/L | 20 (1-727) | 20 (1-727) | 21 (1-324) |
| ANC, × 109/L | 3.0 (0.2-15.2) | 3.0 (0.2-14.3) | 2.8 (0.2-15.2) |
| LDH, ×ULN | 1.7 (0.5-16) | 1.7 (0.6-16) | 1.5 (0.5-11) |
| IgG, g/dL | 1002 (52-5660) | 1030 (52-5660) | 862 (113-1440) |
| IgA, g/dL | 155 (24-1257) | 145 (24-1257) | 143 (40-375) |
| IgM, g/dL | 88 (10-1800) | 83 (10-1800) | 88 (27-475) |
| DAT+ | 103 (89) | 80 (87) | 23 (96) |
| IgG | 64 (62) | 48 (60) | 16 (70) |
| IgG + C | 25 (24) | 20 (25) | 5 (22) |
| C | 5 (5) | 5 (6.25) | 0 (0) |
| Mixed | 7 (7) | 6 (7.5) | 1 (4) |
| Atypical | 2 (2) | 1 (1.25) | 1 (4) |
| DAT− AIHA* | 6 (5) | 5 (5) | 1 (4) |
| Positive anti-PLTs (n = 49) | 41 (84) | 37(82)† | 4 (100) |
| Positive antineutrophils (n = 42) | 10 (45) | 8 (42)‡ | 2 (67) |
| ANA (n = 94) | 34 (36) | 29/74 | 5/20 |
| ENA (n = 69) | 6 (7) | 4/57 | 2/12 |
| Anti-DNA (n = 72) | 4 (56) | 2/58 | 2/14 |
| Anticardiolipin antibodies (n = 70) | 12 (17) | 11/56 | 1/14 |
| Anti–β2 glycoprotein 1 antibodies (n = 51) | 9 (18) | 7/46 | 2/5 |
| LAC (n = 60) | 9 (15) | 4/49 | 5/11 |
| Disease categories | |||
| Cytopenia at onset | |||
| ITP | 39 (34) | 34 (37) | 5 (21) |
| AIHA | 36 (31) | 30 (33) | 6 (25) |
| AIN | 5 (4) | 4 (4) | 1 (4) |
| Concomitant | 36 (31) | 24 (26) | 12 (50) |
| ES type | |||
| ITP and AIHA | 94 (81) | 75 (82) | 19 (79) |
| ITP and AIN | 7 (6) | 6 (6) | 1 (4) |
| AIHA and AIN | 3 (3) | 3 (3) | 0 (0) |
| ITP, AIHA, and AIN | 12 (10) | 8 (9) | 4 (17) |
| Splenomegaly | 37 (32) | 26 (28) | 11 (46) |
| Complications and outcomes | |||
| Infection | 39 (34) | 26 (28) | 13 (54) |
| Thrombosis | 25 (22) | 17 (18) | 8 (33) |
| Death§ | 23 (21) | 19 (21) | 4 (17) |
Demographics and laboratory data reflect those registered at first ES diagnosis. Values are given as median (range) or n (%).
ANA, antinuclear antibody; ENA, extractable nuclear antigen; LDH, lactate dehydrogenase; ULN, upper limit of normal.
Patients with DAT− AIHA were diagnosed after exclusion of all other causes of hemolytic anemia and response to steroids; percentages calculated based on 109 total AIHA cases.
n = 45 tested.
n = 19.
6 patients lost to follow-up.