Table 1 Patients characteristics and transplant outcome.

From: Poems syndrome: consolidation with autologous stem cell transplantation

Parameter

Value

Age at diagnosis (years), median (range)

42 (26–59)

Sex (M:F)

16:3

ECOG PS

N = 19

2

5 (26.3%)

3

7 (36.8%)

4

7 (36.8%)

Interval from diagnosis to transplant median (range)

11.9 (4.9-75.6)

POEMS syndrome marker

N (%)

Polyneuropathy

19 (100)

Monoclonal Plasmacytosis

18 (94.7)

Castleman Disease

6 (31.5)

*Hyaline vascular

5/6

*Plasma cell variant

1/6

#multicentric

5/6

Sclerotic bone lesion

12 (63.1)

Organomegaly

9 (47.3)

Lymphadenopathy

9 (47.3)

Skin Changes

12 (63.1)

Endocrinopathy

10 (52.6)

Gonadal dysfunction

9 (47.3)

Thyroid dysfunction

5 (26.3)

Adrenal dysfunction

5 (26.3)

Gynecomastia

3 (15.7)

Extravascular Volume Overload

11 (57.8)

Papilledema

7 (36.8)

Clubbing

4 (21)

Thrombocytosis (>4.5lakhs)

3 (15.7)

Weight loss >5kg-

6 (31.5)

Fatigue

11(57.8)

Ig Characteristic (Heavy & light chain)

 IgG Lambda

8(42.1)

 IgG Kappa

4 (21)

 IgG Kappa + Lambda

1 (5)

 Ig A Lambda

1 (5)

 Lambda light chain

1 (5)

 Kappa light chain

2 (10.5)

 Focal

1 (5)

 Negative

1 (5)

Bone marrow involvement

  > =10%

3 (15.7)

 5–9%

7 (36.8)

  < 5%

9 (47.2)

Induction

Vd

11 (57.8)

VRd

4 (21.05)

 Rd

2 (10.5)

RVCd

1 (5.26)

 MP

1 (5.26

Response after induction (median 6 cycles)

 CHR

13 (68.4)

 PHR

4 (21.05)

 Stable

2 (10.5)

Stem cell mobilisation:

 G-CSF

13 (68.4%)

 G-CSF + Plerixafor

6 (31.5%)

 Median stem cell dose, x 106 CD34 cells/kg

2.64(1.83-7.05)

High dose Melphalan:

•140 mg/m2

11 (57.8%)

•160 mg/m2

2(10.5%)

•200 mg/ m2

6(31.5%)

Hematological Response (median 6 cycles received)

Post-Induction

Pre -ASCT

Post ASCT- D + 100

 CR

13

15

 19(100%)

 PR

4

4

 

 Stable

4

0

 

Neurological Response

 CR

0

0

1

 PR

16

12

16

 SD

2

5

2

 PD

1

0

0

mRS

   

 1

0

2

3

 2

4

4

9

 3

12

10

7

 4

3

3

0

Overall Clinical response

 

Partial Response

Partial response

  1. CHR Complete hematological response, PHR Partial hematological Response, Vd bortezomib + dexamethasone, VRd bortezomib,+ lenalidomide + dexamethasone, Rd lenalidomide+dexamethasone, VCd bortezomib,+ cyclophosphamide + dexamethasone, MP melphalan+prednisolone, CR complete response, PR partial response, SD stable disease, mRS modified rankin score.