Management of nonsevere scleromyxedema
| Lines of therapy . | Treatment . | Mean dose . | Mean follow-up duration, mo . | Response, n . | Failure, n (%) . | DNS/cardiac injury or death, n . | Severe side effects (≥grade 3-4), n . |
|---|---|---|---|---|---|---|---|
| First line (n = 32) | HDIVig alone (n = 15) | 2 g/kg/m | 21 | 10 CR/5 PR | 2 (13) | 4 | 1 thrombosis |
| HDIVig + CTs (n = 5) | 2 g/kg/mo + 1 mg/kg/d | 10.2 | 5 PR | 1 (20) | 0 | 0 | |
| Thalidomide (n = 3) | 100 mg/d | 33.2 | 1 PR/2 NR | 2 (66) | 0 | 1 neuropathy | |
| Lenalidomide (n = 1) | 25 mg/d (21 d/28) | 11 | 1 NR | 1 (100) | 0 | 0 | |
| Acitretin (n = 2) | 35 mg/d | 8 | 2 NR | 2 (100) | 0 | 0 | |
| CTs alone* (n = 3) | 0.75 mg/kg/d | 6 | 1 CR/2 NR | 0 | 0 | 0 | |
| Melphalan + dexamethasone (n = 2) | 10 mg/m2 PO on days 1-4 + 40 mg on days 1-4 | 0.1 | 1 PR/1 NR | 2 (100) | 0 | 1 severe sepsis | |
| Methotrexate (n = 1) | 15 mg/wk | 3 | 1 NR | 1 (100) | 0 | 0 | |
| Second line (n = 11) | HDIVig (n = 6) | 2 g/kg/mo | 24 | 2 CR/4 PR | 1 (17) | 1 | 0 |
| HDIVig + CTs (n = 3) | 2 g/kg/mo + 0.3 mg/kg/d | 13 | 1 CR /2 PR | 0 | 0 | 0 | |
| Thalidomide + CTs (n = 1) | 100 mg/d + 0.25 mg/kg/d | 1 | 1 PR | 1 (100) | 0 | 1 neuropathy | |
| Lenalidomide (n = 1) | 25 mg/d (21 d/28) | 21 | 1 PR | 1 (100) | 0 | 0 | |
| Third line and more (n = 9) | HDIVig (n = 4) | 2 g/kg/mo | 28 | 4 PR | 0 | 0 | 0 |
| HDIVig + CT (n = 2) | 2 g/kg/mo + 0.5 mg/kg/d | 22.5 | 1 CR/1 PR | 0 | 0 | 0 | |
| HDIVig + lenalidomide (n = 1) | 2 g/kg/mo + 25 mg/d (21 d/28) | 16 | 1 CR | 0 | 0 | 0 | |
| HDIVig + thalidomide (n = 1) | 2 g/kg/mo + 100 mg/d | 3 | 1 PR | 0 | 0 | 0 | |
| Lenalidomide (n = 1) | 25 mg/d (21 d/28) | 4.73 | 1 PR | 0 | 0 | 0 |
| Lines of therapy . | Treatment . | Mean dose . | Mean follow-up duration, mo . | Response, n . | Failure, n (%) . | DNS/cardiac injury or death, n . | Severe side effects (≥grade 3-4), n . |
|---|---|---|---|---|---|---|---|
| First line (n = 32) | HDIVig alone (n = 15) | 2 g/kg/m | 21 | 10 CR/5 PR | 2 (13) | 4 | 1 thrombosis |
| HDIVig + CTs (n = 5) | 2 g/kg/mo + 1 mg/kg/d | 10.2 | 5 PR | 1 (20) | 0 | 0 | |
| Thalidomide (n = 3) | 100 mg/d | 33.2 | 1 PR/2 NR | 2 (66) | 0 | 1 neuropathy | |
| Lenalidomide (n = 1) | 25 mg/d (21 d/28) | 11 | 1 NR | 1 (100) | 0 | 0 | |
| Acitretin (n = 2) | 35 mg/d | 8 | 2 NR | 2 (100) | 0 | 0 | |
| CTs alone* (n = 3) | 0.75 mg/kg/d | 6 | 1 CR/2 NR | 0 | 0 | 0 | |
| Melphalan + dexamethasone (n = 2) | 10 mg/m2 PO on days 1-4 + 40 mg on days 1-4 | 0.1 | 1 PR/1 NR | 2 (100) | 0 | 1 severe sepsis | |
| Methotrexate (n = 1) | 15 mg/wk | 3 | 1 NR | 1 (100) | 0 | 0 | |
| Second line (n = 11) | HDIVig (n = 6) | 2 g/kg/mo | 24 | 2 CR/4 PR | 1 (17) | 1 | 0 |
| HDIVig + CTs (n = 3) | 2 g/kg/mo + 0.3 mg/kg/d | 13 | 1 CR /2 PR | 0 | 0 | 0 | |
| Thalidomide + CTs (n = 1) | 100 mg/d + 0.25 mg/kg/d | 1 | 1 PR | 1 (100) | 0 | 1 neuropathy | |
| Lenalidomide (n = 1) | 25 mg/d (21 d/28) | 21 | 1 PR | 1 (100) | 0 | 0 | |
| Third line and more (n = 9) | HDIVig (n = 4) | 2 g/kg/mo | 28 | 4 PR | 0 | 0 | 0 |
| HDIVig + CT (n = 2) | 2 g/kg/mo + 0.5 mg/kg/d | 22.5 | 1 CR/1 PR | 0 | 0 | 0 | |
| HDIVig + lenalidomide (n = 1) | 2 g/kg/mo + 25 mg/d (21 d/28) | 16 | 1 CR | 0 | 0 | 0 | |
| HDIVig + thalidomide (n = 1) | 2 g/kg/mo + 100 mg/d | 3 | 1 PR | 0 | 0 | 0 | |
| Lenalidomide (n = 1) | 25 mg/d (21 d/28) | 4.73 | 1 PR | 0 | 0 | 0 |
PO, by mouth.
Prednisone equivalent dose.