Activity of immune modulatory drug therapies for the treatment of POEMS syndrome
| Drug therapy . | Outcome . |
|---|---|
| Thal after MP67 | No hematologic response but improved ascites; stabilized PN, splenomegaly, pulmonary hypertension |
| Thal + Dex after CAD68 | CD/POEMS: improved ascites, effusions, pulmonary hypertension, PN, renal function, IL-6 level |
| Thal + Dex69 | 9 patients: VEGF improved in all; PN improved in 66%; stable in 33%; improved edema; no HCR |
| Thal after VAD, CTX, Bev25 | Improved cardiopulmonary status, but no improved PN and rising VEGF |
| Len + Dex63 | Improved ascites, PS, PN, VEGF, testosterone, pulmonary function tests |
| Len + Dex64 | 9 patients: all had hematologic response; clinical responses in all evaluable patients, including PS, neurologic syndrome, edema, and VEGF |
| Len ± Dex65 | 10 patients: all had prior therapy a median of 4 mo (range, 1-36 mo) before starting Len; for 7, only Pred and IVIG were used as prior therapy, making it improbable that the salutatory effect was related to anything other than Len; after a median of 7.5 cycles of Len, all had clinical improvement despite the fact that only half achieved CR; 5 were consolidated with ASCT |
| Len + CTX + Dex66 | After 4 cycles of therapy, patient was able to walk without support; and after 6 cycles, papilledema and IgA disappeared; 1 y after 9 cycles, she remains in remission |
| Drug therapy . | Outcome . |
|---|---|
| Thal after MP67 | No hematologic response but improved ascites; stabilized PN, splenomegaly, pulmonary hypertension |
| Thal + Dex after CAD68 | CD/POEMS: improved ascites, effusions, pulmonary hypertension, PN, renal function, IL-6 level |
| Thal + Dex69 | 9 patients: VEGF improved in all; PN improved in 66%; stable in 33%; improved edema; no HCR |
| Thal after VAD, CTX, Bev25 | Improved cardiopulmonary status, but no improved PN and rising VEGF |
| Len + Dex63 | Improved ascites, PS, PN, VEGF, testosterone, pulmonary function tests |
| Len + Dex64 | 9 patients: all had hematologic response; clinical responses in all evaluable patients, including PS, neurologic syndrome, edema, and VEGF |
| Len ± Dex65 | 10 patients: all had prior therapy a median of 4 mo (range, 1-36 mo) before starting Len; for 7, only Pred and IVIG were used as prior therapy, making it improbable that the salutatory effect was related to anything other than Len; after a median of 7.5 cycles of Len, all had clinical improvement despite the fact that only half achieved CR; 5 were consolidated with ASCT |
| Len + CTX + Dex66 | After 4 cycles of therapy, patient was able to walk without support; and after 6 cycles, papilledema and IgA disappeared; 1 y after 9 cycles, she remains in remission |
Thal indicates thalidomide; MP, PN, polyneuropathy; Dex, dexamethasone; CAD, cyclophosphamide, doxorubicin, and dexamethasone; CD, Castleman disease; HCR, hematologic complete response; VAD, vincristine, doxorubicin, dexamethasone; CTX, cyclophosphamide; Bev, bevacizumab; Len, lenalidomide; PS, performance status; and Pred, prednisone.