Characteristics of 7 severe or HDIVig-refractory scleromyxedema patients treated with plasma cell–directed therapies
| Patient no. . | Age, y . | Sex . | Characteristics . | MG, level; hemopathy . | Previous treatment . | Status . | Treatment . | Duration/protocol . | Clinical response . | Hematologic response . |
|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 65 | F | PE, myalgia, and arthralgia | κ, 4 g/L; MGUS | HDIVig | HDIVig-refractory disease | Bortezomib + cyclophosphamide + dexamethasone and HDIVig | 4 mo* | PR | CR (IF negative) |
| 2 | 61 | F | PE, CTS, and arthralgia | λ, 4 g/L; MGUS | HDIVig | HDIVig- refractory disease | Bortezomib + melphalan + dexamethasone | 1 mo† | NR, death | NR |
| 3 | 50 | M | PE, recurrent DNS, and CTS | κ, 3.9 g/L; MGUS | HDIVig | HDIVig-refractory and severe disease | Bortezomib + cyclophosphamide + dexamethasone | 2 mo* | NR | PR |
| Bortezomib + lenalidomide + dexamethasone | 5 mo‡ | PR (almost complete) | CR (IF negative) | |||||||
| 4 | 43 | M | PE, DNS, and CTS | λ, 4.5 g/L; smoldering myeloma | HDIVig + CT | HDIVig-refractory disease | Lenalidomide + dexamethasone + HDIVig | 6 mo§ | PR (almost complete) | CR (IF negative) |
| 5 | 63 | F | PE, DNS, epilepsy, and ischemic cardiopathy | κ, 11 g/L; MGUS | — | Severe disease | Bortezomib + dexamethasone | 4 mo¶ | CR | CR (IF negative) |
| 6 | 29 | F | PE, DNS, livedo, and GAL | λ, 7 g/L; MGUS | HDIVig | HDIVig-refractory and severe disease | Lenalidomide + dexamethasone + HDIVig | 3 mo§ | PR | CR (IF negative) |
| 7 | 28 | F | PE, dilated cardiomyopathy, and impaired consciousness | λ, 5.3 g/L; MGUS | HDIVig | HDIVig-refractory and severe disease | Lenalidomide + dexamethasone + HDIVig | 3 mo§ | PR | CR (IF negative) |
| Patient no. . | Age, y . | Sex . | Characteristics . | MG, level; hemopathy . | Previous treatment . | Status . | Treatment . | Duration/protocol . | Clinical response . | Hematologic response . |
|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 65 | F | PE, myalgia, and arthralgia | κ, 4 g/L; MGUS | HDIVig | HDIVig-refractory disease | Bortezomib + cyclophosphamide + dexamethasone and HDIVig | 4 mo* | PR | CR (IF negative) |
| 2 | 61 | F | PE, CTS, and arthralgia | λ, 4 g/L; MGUS | HDIVig | HDIVig- refractory disease | Bortezomib + melphalan + dexamethasone | 1 mo† | NR, death | NR |
| 3 | 50 | M | PE, recurrent DNS, and CTS | κ, 3.9 g/L; MGUS | HDIVig | HDIVig-refractory and severe disease | Bortezomib + cyclophosphamide + dexamethasone | 2 mo* | NR | PR |
| Bortezomib + lenalidomide + dexamethasone | 5 mo‡ | PR (almost complete) | CR (IF negative) | |||||||
| 4 | 43 | M | PE, DNS, and CTS | λ, 4.5 g/L; smoldering myeloma | HDIVig + CT | HDIVig-refractory disease | Lenalidomide + dexamethasone + HDIVig | 6 mo§ | PR (almost complete) | CR (IF negative) |
| 5 | 63 | F | PE, DNS, epilepsy, and ischemic cardiopathy | κ, 11 g/L; MGUS | — | Severe disease | Bortezomib + dexamethasone | 4 mo¶ | CR | CR (IF negative) |
| 6 | 29 | F | PE, DNS, livedo, and GAL | λ, 7 g/L; MGUS | HDIVig | HDIVig-refractory and severe disease | Lenalidomide + dexamethasone + HDIVig | 3 mo§ | PR | CR (IF negative) |
| 7 | 28 | F | PE, dilated cardiomyopathy, and impaired consciousness | λ, 5.3 g/L; MGUS | HDIVig | HDIVig-refractory and severe disease | Lenalidomide + dexamethasone + HDIVig | 3 mo§ | PR | CR (IF negative) |
CT, corticosteroids PO; CTS, carpal tunnel syndrome; IF, immunofixation; IVP, intravenous pyelogram; livedo, livedo racemosa; PE, papular eruption.
Bortezomib, 1.3 mg/m2 IVP on days 1, 4, 8, and 11 + cyclophosphamide, 300 mg/m2 per day PO on days 1, 8, 15, and 22 + dexamethasone 40 mg PO on days 1, 8, 15, and 22.
Melphalan, 10 mg/m2 PO on days 1 through 4 + bortezomib, 1.3 mg/m2 IVP on days 8, 15, and 22 + oral dexamethasone, 40 mg/d on days 1 through 4.
Bortezomib, 1.3 mg/m2 IVP on days 1, 4, 8, and 11 + lenalidomide, 25 mg/d on days 1 through 14 + dexamethasone, 20 mg/d on days 1, 2, 4, 5, 8, 9, 11, and 12.
Lenalidomide, 25 mg/d on days 1 through 21 + dexamethasone, PO 40 mg on days 1, 8, 15, and 22 + HDIVig, 2 g/kg per month.
Bortezomib, 1.3 mg/m2 IVP on days 1, 4, 8, and 11 + dexamethasone, 40 mg on days 1, 2, 3, 4, 8, 9, 10, and 11.