Description of cGVHD
| . | All, n (%) . | Hypergammaglobulinemia, n (%) . | Hypogammaglobulinemia, n (%) . | Normogammaglobulinemia, n (%) . |
|---|---|---|---|---|
| No. of patients | 76 | 25 | 21 | 30 |
| Organ involvement at study entry | ||||
| Skin | 47 (62) | 16 (64) | 15 (71) | 16 (53) |
| Deep cutaneous sclerosis | 17 (22) | 11 (44) | 6 (29) | 0 |
| Eyes | 26 (34) | 11 (44) | 13 (62) | 2 (6) |
| Oral mucosa | 43 (57) | 17 (68) | 12 (57) | 14 (47) |
| Liver | 24 (32) | 13 (52) | 7 (33) | 4 (13) |
| Lungs | 21 (28) | 10 (40) | 10 (48) | 1 (3) |
| GI | 6 (8) | 2 (8) | 3 (14) | 1 (3) |
| Genital* | 7 (20) | 2 (22) | 3 (33) | 2 (12) |
| Joints | 11 (14) | 5 (20) | 3 (14) | 3 (10) |
| Onset type | ||||
| De novo | 33 (44) | 11 (44) | 6 (28) | 16 (53) |
| Quiescent | 26 (34) | 9 (36) | 9 (43) | 8 (27) |
| Progressive | 17 (22) | 5 (20) | 6 (28.5) | 6 (20) |
| NIH severity score | ||||
| Mild | 7 (9) | 3 (12) | 1 (5) | 3 (10) |
| Moderate | 19 (25) | 5 (20) | 2 (9) | 12 (40) |
| Severe | 50 (66) | 17 (68) | 18† (86) | 15† (50) |
| No. of organs involved | ||||
| ≤ 2 | 27 (36) | 11 (44) | 4 (19) | 12 (40) |
| > 2 | 49 (64) | 14 (56) | 17 (81) | 18 (60) |
| Median duration of cGVHD | ||||
| mo | 42 | 29‡ | 45 | 63‡ |
| (range) | (3-136) | (3-92) | (3-136) | (26-110) |
| Immunosuppressive therapy | ||||
| CSA/FK with or without MMF with or without steroids | 18 (24) | 3 (12) | 7 (33) | 8 (27) |
| ECP with or without steroids | 12 (16) | 7 (28) | 5 (24) | 0 |
| Sirolimus with or without steroids | 12 (16) | 5 (20) | 7 (33) | 0 |
| Steroids alone | 21 (28) | 9 (36) | 2 (10) | 10 (33) |
| No systemic therapy | 13 (17) | 1 (4) | 0 | 12 (40) |
| No. of IS therapies before study entry | ||||
| 1 | 21 (28) | 7 (28) | 2 (10) | 12 (40) |
| 2 | 25 (33) | 7 (28) | 4 (19) | 14 (47) |
| ≥ 3 | 30 (39) | 11 (44) | 15 (71) | 4 (13) |
| Mean IgG, mg/dL | 1441 | 3040 | 226 | 960 |
| (range) | (100-3950) | (2320-3950) | (100-395) | (750-1300) |
| Mean IgM, mg/dL | 87 | 127 | 52 | 78 |
| (range) | (12-507) | (36-507) | (12-84) | (16-133) |
| Mean IgA, mg/dL | 104 | 141 | 80 | 108 |
| (range) | (6-735) | (17-735) | (6-155) | (6-273) |
| No. of patients with autoantibodies present | 24 (32) | 17§ (68) | 5§ (24) | 2 (7) |
| No. of patients with autoantibodies absent | 52 (68) | 8 (32) | 16 (76) | 28 (93) |
| No. of patients with ANA present | 24 (32) | 17 (68) | 5 (24) | 2 (7) |
| Mean ANA, IU/mL | 764 | 926 | 762 | 320 |
| (range) | (70-1280) | (70-1280) | (320-1280) | (160-320) |
| > 1250 | 10 | 9 | 1 | 0 |
| > 640 ≤ 1250 | 7 | 6 | 1 | 0 |
| ≤ 640 | 2 | 0 | 0 | 2 |
| No. of patients with anti-dsDNA present | 7 (9) | 5 (20) | 1 (5) | 1 (3) |
| Mean anti-dsDNA, IU/mL | 23.5 | 26 | 13 | 13 |
| (range) | (13-66) | (13-66) | NA | NA |
| . | All, n (%) . | Hypergammaglobulinemia, n (%) . | Hypogammaglobulinemia, n (%) . | Normogammaglobulinemia, n (%) . |
|---|---|---|---|---|
| No. of patients | 76 | 25 | 21 | 30 |
| Organ involvement at study entry | ||||
| Skin | 47 (62) | 16 (64) | 15 (71) | 16 (53) |
| Deep cutaneous sclerosis | 17 (22) | 11 (44) | 6 (29) | 0 |
| Eyes | 26 (34) | 11 (44) | 13 (62) | 2 (6) |
| Oral mucosa | 43 (57) | 17 (68) | 12 (57) | 14 (47) |
| Liver | 24 (32) | 13 (52) | 7 (33) | 4 (13) |
| Lungs | 21 (28) | 10 (40) | 10 (48) | 1 (3) |
| GI | 6 (8) | 2 (8) | 3 (14) | 1 (3) |
| Genital* | 7 (20) | 2 (22) | 3 (33) | 2 (12) |
| Joints | 11 (14) | 5 (20) | 3 (14) | 3 (10) |
| Onset type | ||||
| De novo | 33 (44) | 11 (44) | 6 (28) | 16 (53) |
| Quiescent | 26 (34) | 9 (36) | 9 (43) | 8 (27) |
| Progressive | 17 (22) | 5 (20) | 6 (28.5) | 6 (20) |
| NIH severity score | ||||
| Mild | 7 (9) | 3 (12) | 1 (5) | 3 (10) |
| Moderate | 19 (25) | 5 (20) | 2 (9) | 12 (40) |
| Severe | 50 (66) | 17 (68) | 18† (86) | 15† (50) |
| No. of organs involved | ||||
| ≤ 2 | 27 (36) | 11 (44) | 4 (19) | 12 (40) |
| > 2 | 49 (64) | 14 (56) | 17 (81) | 18 (60) |
| Median duration of cGVHD | ||||
| mo | 42 | 29‡ | 45 | 63‡ |
| (range) | (3-136) | (3-92) | (3-136) | (26-110) |
| Immunosuppressive therapy | ||||
| CSA/FK with or without MMF with or without steroids | 18 (24) | 3 (12) | 7 (33) | 8 (27) |
| ECP with or without steroids | 12 (16) | 7 (28) | 5 (24) | 0 |
| Sirolimus with or without steroids | 12 (16) | 5 (20) | 7 (33) | 0 |
| Steroids alone | 21 (28) | 9 (36) | 2 (10) | 10 (33) |
| No systemic therapy | 13 (17) | 1 (4) | 0 | 12 (40) |
| No. of IS therapies before study entry | ||||
| 1 | 21 (28) | 7 (28) | 2 (10) | 12 (40) |
| 2 | 25 (33) | 7 (28) | 4 (19) | 14 (47) |
| ≥ 3 | 30 (39) | 11 (44) | 15 (71) | 4 (13) |
| Mean IgG, mg/dL | 1441 | 3040 | 226 | 960 |
| (range) | (100-3950) | (2320-3950) | (100-395) | (750-1300) |
| Mean IgM, mg/dL | 87 | 127 | 52 | 78 |
| (range) | (12-507) | (36-507) | (12-84) | (16-133) |
| Mean IgA, mg/dL | 104 | 141 | 80 | 108 |
| (range) | (6-735) | (17-735) | (6-155) | (6-273) |
| No. of patients with autoantibodies present | 24 (32) | 17§ (68) | 5§ (24) | 2 (7) |
| No. of patients with autoantibodies absent | 52 (68) | 8 (32) | 16 (76) | 28 (93) |
| No. of patients with ANA present | 24 (32) | 17 (68) | 5 (24) | 2 (7) |
| Mean ANA, IU/mL | 764 | 926 | 762 | 320 |
| (range) | (70-1280) | (70-1280) | (320-1280) | (160-320) |
| > 1250 | 10 | 9 | 1 | 0 |
| > 640 ≤ 1250 | 7 | 6 | 1 | 0 |
| ≤ 640 | 2 | 0 | 0 | 2 |
| No. of patients with anti-dsDNA present | 7 (9) | 5 (20) | 1 (5) | 1 (3) |
| Mean anti-dsDNA, IU/mL | 23.5 | 26 | 13 | 13 |
| (range) | (13-66) | (13-66) | NA | NA |
GI indicates gastrointestinal; NIH, National Institutes of Health; CSA, cyclosporine A; FK, tacrolimus; ECP, extracorporeal photopheresis; MMF, mycophenolate mofetil; IS, immunosuppressive; ANA, antinuclear antibody; anti-dsDNA, antibody against double-stranded DNA; and NA, not applicable.
Only female patients.
P = .04.
P < .0001.
P = .024.