Activity of proteosome inhibitors for the treatment of POEMS syndrome
| Treatment . | Outcome . |
|---|---|
| Bortez + AD after VAD, CTX, Mel-Pred, + AD70 | Improved M-protein, VEGF, paresthesias, splenomegaly, effusions, muscle strength, gynecomastia, and skin changes |
| Bortez + Dex71 | Improved M-protein, polyneuropathy, hepatomegaly, testosterone; no change in electromyelography |
| Bortez × 5 cycles + Thal added at cycle 6 (prior Dex and Mel-Pred)72 | Improvement of anasarca, PN, VEGF, and PET scan with Bortez alone, but Thal added because of persistent edema, M-protein, PN, and barely elevated VEGF; with Thal, disappearance of pleural effusion, ascites, and M-protein and normalization of VEGF |
| Bortez Dex*73 | Improvement by 3 cycles, but continued for 6; complete remission 4 y after completing therapy; improvement in adenopathy, pleural effusion and ascites, hepatosplenomegaly, and IL-6 |
| Bortez, CTX, Dex74 | Clinical response of anasarca within 6 wks and tolerated therapy for 18 mo achieving a nCR and a VEGF response; PN, hyperpigmentation, pulmonary hypertension improved significantly |
| Treatment . | Outcome . |
|---|---|
| Bortez + AD after VAD, CTX, Mel-Pred, + AD70 | Improved M-protein, VEGF, paresthesias, splenomegaly, effusions, muscle strength, gynecomastia, and skin changes |
| Bortez + Dex71 | Improved M-protein, polyneuropathy, hepatomegaly, testosterone; no change in electromyelography |
| Bortez × 5 cycles + Thal added at cycle 6 (prior Dex and Mel-Pred)72 | Improvement of anasarca, PN, VEGF, and PET scan with Bortez alone, but Thal added because of persistent edema, M-protein, PN, and barely elevated VEGF; with Thal, disappearance of pleural effusion, ascites, and M-protein and normalization of VEGF |
| Bortez Dex*73 | Improvement by 3 cycles, but continued for 6; complete remission 4 y after completing therapy; improvement in adenopathy, pleural effusion and ascites, hepatosplenomegaly, and IL-6 |
| Bortez, CTX, Dex74 | Clinical response of anasarca within 6 wks and tolerated therapy for 18 mo achieving a nCR and a VEGF response; PN, hyperpigmentation, pulmonary hypertension improved significantly |
Bortez indicates bortezomib; AD, doxorubicin and dexamethasone; VAD, vincristine, doxorubicin, dexamethasone; CTX, cyclophosphamide; Mel, mephalan; Pred, prednisone; Dex, dexamethasone; PN, polyneuropathy; Thal, thalidomide; and nCR, near complete response.
Castleman variant of POEMS syndrome.