Table 1.
Patient | Age | Months post HSCT | Months post cGVHD diagnosis | Systemic immune suppression at the time of evaluationa | NIH global cGVHD severity scoreb | Number of organ systems affected | Oral cGVHDc,d | Skind,e | PBMC Tregf | Total % BSAg erythema | Total % BSAg sclerosis |
---|---|---|---|---|---|---|---|---|---|---|---|
1 | 53 | 13 | 7 | GC, TL, MMF, ECP | Severe | 4 | Severe | Lichenoid | FACS | 30 | 0 |
2 | 50 | 9 | 2 | GC, CSA | Moderate | 4 | Severe | Lichenoid | FACS | 21 | 0 |
3 | 47 | 6 | 2 | GC | Moderate | 3 | Severe | Lichenoid | FACS | 5 | 0 |
4 | 22 | 9 | 3 | GC, TL, MMF | Moderate | 4 | Severe | (Lichenoid) | FACS | 26 | 0 |
5 | 57 | 46 | 33 | GC, TL, MMF, ECP | Severe | 5 | Severe | (Sclerotic) | FACS | 0 | 51 |
6 | 40 | 55 | 24 | GC, MMF, SL, R | Severe | 4 | Severe | Sclerotic | 8 | 49 | |
7 | 56 | 28 | 10 | GC, TL, MMF | Moderate | 4 | Severe | Lichenoid | 18 | 0 | |
8 | 51 | 64 | 61 | GC, CSA, MMF | Severe | 6 | Severe | (Lichenoid) | 3 | 0 | |
9 | 45 | 6 | 3 | CSA | Moderate | 6 | (Severe) | Lichenoid | FACS | 1 | 0 |
10 | 58 | 5 | 3 | Noneh | Moderate | 2 | Severe | Lichenoid | FACS | 0 | 0 |
11 | 41 | 147 | 135 | Noneh | Severe | 5 | Mild | Sclerotic | 0 | 30 | |
12 | 44 | 33 | 28 | CSA, MMF | Moderate | 3 | Mild | None | 0 | 0 | |
13 | 39 | 58 | 56 | GC, TL, HCQ | Severe | 5 | Mild | Sclerotic | FACS | 5 | 70 |
14 | 44 | 205 | 198 | MMF, CSA, ECP | Severe | 7 | Mild | Sclerotic | 0 | 30 | |
15 | 42 | 100 | 92 | GC | Moderate | 3 | Mild | (None) | 0 | 0 | |
16 | 59 | 22 | 13 | GC, TL | Severe | 4 | Mild | Sclerotic | FACS | 0 | 20 |
17 | 36 | 41 | 35 | MMF | Moderate | 5 | Mild | (None) | 0 | 1 | |
18 | 36 | 29 | 19 | Noneh | Moderate | 4 | Mild | (None) | 0 | 0 | |
19 | 53 | 159 | 147 | GC, CSA | Severe | 3 | Mild | (None) | 0 | 0 | |
20 | 55 | 5 | 3 | GC, CSA | Moderate | 6 | Mild | Lichenoid | FACS | 41 | 0 |
21 | 39 | 55 | 43 | TL, MMF, ECP | Severe | 6 | Mild | Sclerotic | 1 | 45 | |
22 | 52 | 12 | 8 | CSA | Moderate | 6 | Mild | Lichenoid | 6 | 0 | |
23 | 58 | 29 | 23 | Noneh | Severe | 3 | Mild | (None) | 0 | 0 | |
24 | 45 | 27 | 20 | GC | Severe | 5 | Mild | Sclerotic | FACS | 2 | 64 |
25 | 60 | 43 | 25 | GC, TL, MMF | Moderate | 6 | Mild | (Sclerotic) | 0 | 5 | |
26 | 56 | 27 | 0 | Noneh | Moderate | 3 | None | Sclerotic | 0 | 29 | |
27 | 61 | 39 | 35 | Noneh | Mild | 2 | None | None | 0 | 0 | |
28 | 39 | 59 | 34 | TL, MMF | Severe | 6 | None | (None) | 0 | 0 | |
29 | 41 | 12 | 0 | GC, MMF, CSA | Mild | 2 | None | None | 0 | 0 | |
30 | 49 | 28 | 15 | Noneh | Moderate | 3 | None | None | 0 | 0 | |
31 | 40 | 22 | 16 | GC, CSA | Severe | 6 | None | Lichenoid | 7 | 0 | |
32 | 62 | 11 | 6 | Noneh | Moderate | 5 | (None) | Lichenoid | FACS | 10 | 0 |
33 | 58 | 3 | 6 | MMF | Moderate | 4 | (Mild) | Lichenoid | FACS | 5 | 0 |
34 | 58 | 53 | 49 | GC, MMF, ECP | Severe | 2 | (Mild) | Sclerotic | FACS | 0 | 78 |
35 | 51 | 22 | 15 | TL, GC | Severe | 3 | (None) | Sclerotic | FACS | 0 | 62 |
36 | 42 | 48 | 44 | TL, Imatinib | Severe | 5 | (Mild) | (Sclerotic) | FACS | 1 | 81 |
37 | 59 | 24 | 9 | ECP, GC | Severe | 5 | (None) | (Sclerotic) | FACS | 2 | 74 |
38 | 34 | 13 | 9 | TL, GC, Imatinib | Severe | 7 | (Mild) | (Sclerotic) | FACS | 0 | 62 |
39 | 36 | 40 | 6 | TL, MMF, ECP | Severe | 5 | (Mild) | (Sclerotic) | FACS | 1 | 68 |
40 | 24 | 41 | 21 | TL, Imatinib | Severe | 4 | (None) | (Sclerotic) | FACS | 5 | 62 |
Abbreviations: BSA, body surface area; cGVHD, chronic graft versus host disease; CSA, cyclosporine A; ECP, extracorporeal photopheresis; GC, prednisone or other glucocorticoid; HSCT, hematopoietic stem cell transplantation; MMF, mycophenolate mofetil; PBMC, peripheral blood mononuclear cell; R, rituximab; SL, sirolimus; TL, tacrolimus.
Current systemic immunosuppression regimen.
NIH score: Mild—only one or two organs (excluding lungs) with a maximum NIH score of 1 in all organs; Moderate—at least one organ with a score of 2 or lungs with a score of 1, or three or more organs with a max score of 1; Severe—lung score of 2 or any other organ system scoring 3.
Oral cGVHD was evaluated and graded by an oral medicine specialist using the following severity scoring system: normal mucosa, none; hyperkeratotic, lichenoid changes and/or erythema, mild; lichenoid changes and presence of ulcerations, severe.
All patients were evaluated clinically and biopsies were reviewed for GVHD pathology, but biopsies that were not further examined by IHC for FoxP3 are listed in parentheses.
Cutaneous cGVHD was evaluated and graded by an experienced dermatologist as having no evidence of cutaneous cGVHD (None), involving primarily lichen planus-like epidermal changes (Lichenoid) or involving primarily dermal or fascial sclerotic changes (Sclerotic).
Patients with severe lichenoid oral or cutaneous cGVHD (or both) whose cryopreserved PBMC were examined for FoxP3+ Treg subpopulations are noted as FACS.
The percentages of the BSA affected by cutaneous cGVHD are noted.
Three of the eight not currently on therapy had received no prior systemic treatment, but only patient 26 was newly diagnosed with cGVHD.