Comparison of patients in our series to those in the published literature
Patient no. (ref. no.) . | Pretransplantation features . | . | . | . | . | . | . | . | . | . | . | Response . | . | . | ||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
. | Age, y/sex . | Polyneuropathy . | Organomegaly . | Endocrinopathy . | M protein . | Skin changes . | Bone . | Elevated platelets . | Ed/Ef . | Papilledema/Thrombosis . | Rx/TTTd, mo/TTTs, mo . | Follow-up after SCT, mos . | PCD . | Comments . | ||||||||||||
| 1 (4) | 44/M | SM | H | + | + | + | > 3 | − | − | −/− | PE, MP, RT/30/35 | 56 | CR | None | ||||||||||||
| 2 | 63/M | SM | S | + | + | + | > 3 | − | Ed | −/CVA | None/3/22 | 3.8 | Inevaluable | Death | ||||||||||||
| 3 | 20/M | SM | − | − | + | − | 1 | +* | − | −/− | RT, AD/5/8 | 32 | CR | Wheelchair to climbing stairs | ||||||||||||
| 4 | 40/M | SM | − | + | + | + | 0 | + | − | −/− | Pred, IVIG, Hydrea/2/16 | 32 | CR | None | ||||||||||||
| 5 | 43/M | SM | − | + | + | − | > 3 | + | − | −/− | RT, Dex/66/78 | 6 | VGPR | Pain improved | ||||||||||||
| 6 | 59/M | SM | − | + | + | + | 1 | − | Ed | −/− | PE, IVIG, Pred, RT/7/32 | 17 | NE | Wheelchair to AFO and occasional cane | ||||||||||||
| 7 | 53/M | M | SA | + | +, Cas | + | 1 | +* | Ed, Ef, A | −/Splenic vein thrombosis | Pred, Rituxan, Thalidomide, Remicade, CSA, IFN/33/73 | 18 | VGPR | None | ||||||||||||
| 8 | 53/M | SM | S | + | + | + | 1 | + | Ed, A | −/CVA | Pred/2/37 | 10 | CR | Pain and function improved | ||||||||||||
| 9 | 37/M | SM | HSA | + | +, Cas | + | > 3 | − | Ed, A | −/− | Steroids, RT/4/41 | 13 | ND | Wheelchair to basketball and jogging | ||||||||||||
| 10 | 50/F | SM | H | − | + | + | > 3 | + | Ed, Ef, A | −/− | PE, IVIG, Imuran, Pred, oral CTX, Hydrea, IFN/149/185 | 10 | ND | Wheelchair to walker, signing name, using spoon | ||||||||||||
| 11 | 53/M | SM | HS | + | + | + | > 3 | + | Ed, Ef, A | −/CVA | Pred, IVIG/2/37 | 7 | NE | None | ||||||||||||
| 12 | 36/M | SM | S | + | + | − | > 3 | − | Ed | −/− | Pred, IVIG/2/9 | 7 | MR | No more AFO | ||||||||||||
| 13 | 51/M | SM | HSA | + | + | + | 1 | + | Ed | −/− | Pred, IVIG, PE, Imuran, MTX, MMF/3/75 | 3 | CR | None | ||||||||||||
| 14 | 59/M | SM | HS | + | + | + | > 3 | − | −/CVA | None/2/26 | 1 | ND | None | |||||||||||||
| 15 | 60/M | SM | HSA | + | + | + | 1 | − | Ed, A | +/− | RT, Pred/7/67 | 3 | VGPR | Wheelchair to walker | ||||||||||||
| 16 | 50/M | SM | S | + | + | + | > 3 | + | Ed | +/− | CTX, Pred/5/9 | 1 | ND | None | ||||||||||||
| 17 (3) | 25/F | SM | + | + | + | + | > 1 | NR | As | +/− | Pred, RT/−/− | 2 | Inevaluable | Death | ||||||||||||
| 18 (8) | 44/F‡ | M | S | − | + | − | > 1 | − | − | +/− | RT, CSA, Imurel, PE/−/− | 58§ | CR | Improved VEGF levels | ||||||||||||
| 19 (8) | 52/M‡ | S | − | + | + | + | > 1 | − | Ed | †/− | None/−/− | 49§ | CR | Improved VEGF levels | ||||||||||||
| 20 (8) | 50/M∥ | S | HSA | + | + | + | 0 | − | Ed | †/− | CTX/−/− | 32§ | VGPR | None | ||||||||||||
| 21 (8) | 62/M | SM | − | + | + | + | > 1 | − | Ed | †/− | PE, RT/−/− | 12§ | CR | None | ||||||||||||
| 22 (8) | 54/M | S | H | + | + | ±¶ | 1 | − | − | −/− | RT/−/− | 12§ | CR | None | ||||||||||||
| 23 (9) | 58/F | SM | − | + | + | + | > 3 | + | − | −/− | VMCP, MP/−/− | 12 | CR | None | ||||||||||||
| 24 (6) | 43/M | SM | − | + | + | + | 1 | + | − | +/− | PE, CS, RT, AMP, CTX-CS/−/25 | 120 | CR | Improved VEGF levels | ||||||||||||
| 25 (6) | 59/F | S | HSA | − | +♯ | + | 3 | +* | − | −/− | RT, CTX-CS/18/− | 6 | CR | Improved VEGF levels | ||||||||||||
| 26 (7) | 56/M | SM | + | + | + | − | 1 | — | − | −/− | IVIG, CS, RT/−/− | 24 | VGPR | None | ||||||||||||
| 27 (7) | 38/F | SM | + | + | + | + | 1 | — | + | +/− | VAMP/5/− | 6 | CR | None | ||||||||||||
| 28 (5)** | 30 | SM | HA | − | −** | − | > 3 | + | Ed/Ef | −/− | MP, Tam, Ifos/−/24 | 72 | Inevaluable | Not documented POEMS | ||||||||||||
Patient no. (ref. no.) . | Pretransplantation features . | . | . | . | . | . | . | . | . | . | . | Response . | . | . | ||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
. | Age, y/sex . | Polyneuropathy . | Organomegaly . | Endocrinopathy . | M protein . | Skin changes . | Bone . | Elevated platelets . | Ed/Ef . | Papilledema/Thrombosis . | Rx/TTTd, mo/TTTs, mo . | Follow-up after SCT, mos . | PCD . | Comments . | ||||||||||||
| 1 (4) | 44/M | SM | H | + | + | + | > 3 | − | − | −/− | PE, MP, RT/30/35 | 56 | CR | None | ||||||||||||
| 2 | 63/M | SM | S | + | + | + | > 3 | − | Ed | −/CVA | None/3/22 | 3.8 | Inevaluable | Death | ||||||||||||
| 3 | 20/M | SM | − | − | + | − | 1 | +* | − | −/− | RT, AD/5/8 | 32 | CR | Wheelchair to climbing stairs | ||||||||||||
| 4 | 40/M | SM | − | + | + | + | 0 | + | − | −/− | Pred, IVIG, Hydrea/2/16 | 32 | CR | None | ||||||||||||
| 5 | 43/M | SM | − | + | + | − | > 3 | + | − | −/− | RT, Dex/66/78 | 6 | VGPR | Pain improved | ||||||||||||
| 6 | 59/M | SM | − | + | + | + | 1 | − | Ed | −/− | PE, IVIG, Pred, RT/7/32 | 17 | NE | Wheelchair to AFO and occasional cane | ||||||||||||
| 7 | 53/M | M | SA | + | +, Cas | + | 1 | +* | Ed, Ef, A | −/Splenic vein thrombosis | Pred, Rituxan, Thalidomide, Remicade, CSA, IFN/33/73 | 18 | VGPR | None | ||||||||||||
| 8 | 53/M | SM | S | + | + | + | 1 | + | Ed, A | −/CVA | Pred/2/37 | 10 | CR | Pain and function improved | ||||||||||||
| 9 | 37/M | SM | HSA | + | +, Cas | + | > 3 | − | Ed, A | −/− | Steroids, RT/4/41 | 13 | ND | Wheelchair to basketball and jogging | ||||||||||||
| 10 | 50/F | SM | H | − | + | + | > 3 | + | Ed, Ef, A | −/− | PE, IVIG, Imuran, Pred, oral CTX, Hydrea, IFN/149/185 | 10 | ND | Wheelchair to walker, signing name, using spoon | ||||||||||||
| 11 | 53/M | SM | HS | + | + | + | > 3 | + | Ed, Ef, A | −/CVA | Pred, IVIG/2/37 | 7 | NE | None | ||||||||||||
| 12 | 36/M | SM | S | + | + | − | > 3 | − | Ed | −/− | Pred, IVIG/2/9 | 7 | MR | No more AFO | ||||||||||||
| 13 | 51/M | SM | HSA | + | + | + | 1 | + | Ed | −/− | Pred, IVIG, PE, Imuran, MTX, MMF/3/75 | 3 | CR | None | ||||||||||||
| 14 | 59/M | SM | HS | + | + | + | > 3 | − | −/CVA | None/2/26 | 1 | ND | None | |||||||||||||
| 15 | 60/M | SM | HSA | + | + | + | 1 | − | Ed, A | +/− | RT, Pred/7/67 | 3 | VGPR | Wheelchair to walker | ||||||||||||
| 16 | 50/M | SM | S | + | + | + | > 3 | + | Ed | +/− | CTX, Pred/5/9 | 1 | ND | None | ||||||||||||
| 17 (3) | 25/F | SM | + | + | + | + | > 1 | NR | As | +/− | Pred, RT/−/− | 2 | Inevaluable | Death | ||||||||||||
| 18 (8) | 44/F‡ | M | S | − | + | − | > 1 | − | − | +/− | RT, CSA, Imurel, PE/−/− | 58§ | CR | Improved VEGF levels | ||||||||||||
| 19 (8) | 52/M‡ | S | − | + | + | + | > 1 | − | Ed | †/− | None/−/− | 49§ | CR | Improved VEGF levels | ||||||||||||
| 20 (8) | 50/M∥ | S | HSA | + | + | + | 0 | − | Ed | †/− | CTX/−/− | 32§ | VGPR | None | ||||||||||||
| 21 (8) | 62/M | SM | − | + | + | + | > 1 | − | Ed | †/− | PE, RT/−/− | 12§ | CR | None | ||||||||||||
| 22 (8) | 54/M | S | H | + | + | ±¶ | 1 | − | − | −/− | RT/−/− | 12§ | CR | None | ||||||||||||
| 23 (9) | 58/F | SM | − | + | + | + | > 3 | + | − | −/− | VMCP, MP/−/− | 12 | CR | None | ||||||||||||
| 24 (6) | 43/M | SM | − | + | + | + | 1 | + | − | +/− | PE, CS, RT, AMP, CTX-CS/−/25 | 120 | CR | Improved VEGF levels | ||||||||||||
| 25 (6) | 59/F | S | HSA | − | +♯ | + | 3 | +* | − | −/− | RT, CTX-CS/18/− | 6 | CR | Improved VEGF levels | ||||||||||||
| 26 (7) | 56/M | SM | + | + | + | − | 1 | — | − | −/− | IVIG, CS, RT/−/− | 24 | VGPR | None | ||||||||||||
| 27 (7) | 38/F | SM | + | + | + | + | 1 | — | + | +/− | VAMP/5/− | 6 | CR | None | ||||||||||||
| 28 (5)** | 30 | SM | HA | − | −** | − | > 3 | + | Ed/Ef | −/− | MP, Tam, Ifos/−/24 | 72 | Inevaluable | Not documented POEMS | ||||||||||||
Patient nos. 1 through 16 are from our study cohort; patient nos. 17 through 28 are patients studied in the published literature. Ed indicates edema; Ef, effusion; Rx, treatments; TTTd, time to transplant from diagnosis; TTTs, time to transplant from symptoms; SCT, stem cell transplant; PCD, plasma cell disorder; M, motor; SM, sensorimotor; H, hepatomegaly; +, present; −, absent; PE, plasmapheresis; MP, melphalan and prednisone; RT, radiation therapy; CR, complete response; S, splenomegaly; CVA, cerebrovascular accident; AD, adriamycin and dexamethasone; Pred, prednisone; IVIG, intravenous gammaglobulin; Hydrea, hydroxyurea; Dex, dexamethasone; VGPR, very good partial response; NE, not evaluable; AFO, ankle foot orthotic; SL, splenomegaly and hepatomegaly; Cas, Castleman disease; A, ascites; CSA, cyclosporin; IFN, interferon; LSA, hepatomegaly, splenomegaly, lymphadenopathy; ND, not done; F, female; CTX, cyclophosphamide; HS, hepatomegaly and splenomegaly; MR, minimal response; MTX, methotrexate; MMF, mycophenolate mofetil; VEGF, vascular endothelial cell-derived growth factor; VMCP, vincristine, mephalan, cyclophosphamide, and prednisone; CS, corticosteroids; (V)AMP, vincristine, adriamycin, and methylprednisolone; Tam, tamoxifen; Ifos, ifosfamide.
Erythrocytosis.
There were 2 patients said to have papilledema, but it was not indicated which 2.
Patients received tandem transplantation.
Follow-up calculated from PBSC collection rather than after transplantation. Median follow-up after transplantation for whole group is 36 months.
Patient also had nephropathy.
Though listed by authors, telangectasia not a recognized skin feature of POEMS.
Monoclonal kappa.
Patient with osteosclerotic myeloma (OSM), but unclear basis of diagnosis since no documentation of a plasma cell proliferative disorder by biopsy or immunofixation.