Single-agent Bortezomib in previously
untreated, symptomatic Multiple Myeloma
(MM): Results of a Phase 2 Multi-center Study
Paul G Richardson,1 Asher Chanan-Khan,2 Robert Schlossman,1 Nikhil
Munshi,
1 Anne Louise Oaklander,3 L Thompson Heffner,4 Hani Hassoun,5
David Avigan,
6 Anthony Amato,1 Kenneth C Anderson1
1
Dana-Farber Cancer Institute, Boston, MA; 2Roswell Park Cancer
Institute, Buffalo, NY;
3Massachusetts General Hospital, Boston, MA;
4
Emory University, Atlanta, GA; 5Memorial Sloan-Kettering Cancer Center,
New York, NY;
6Beth Israel Deaconess Medical Center, Boston, MA

Study Rationale
Bortezomib (BZ): active in relapsed, refractory MM
1­3
In APEX, BZ monotherapy had greater efficacy in patients
(pts) who received 1 prior line of therapy, vs >1 prior line
1
BZ-based combinations active in frontline MM
4­12
Peripheral neuropathy (PN): may require dose modification
and/or discontinuation
1­3,13
­ Evaluate baseline PN, as part of MM
14,15
­ Assess incidence during BZ treatment, manage
appropriately
1. Richardson et al. N Engl J Med 2005;352:2487­98.
9. Orlowski et al. Haematologica 2005;90:151 (Abstract).
2. Richardson et al. N Engl J Med 2003;348:2609­17.
10. Wang et al. Blood 2005;106:Abstract 784.
3. Jagannath et al. Br J Haematol 2004;127:165­72.
11. Badros et al. Blood 2005;106:Abstract 2747.
4. Mateos et al. Blood 2005;106:Abstract 786.
12. Barlogie et al. Blood 2005;106:Abstract 1154.
5. Jagannath et al. Br J Haematol 2005;129:776­83.
13. San Miguel et al. Blood 2005;106:Abstract 366.
6. Jagannath et al. Blood 2005;106:Abstract 783.
14. Dispenzieri et al. Best Pract Res Clin Haematol 2005;
7. Harousseau et al. J Clin Oncol 2005;23:Abstract 6653.
18:673­88.
8. Oakervee et al. Br J Haematol 2005;129:755­62.
15. Ropper et al. N Engl J Med 1998;338:1601­7.

Objectives
Primary
­ Overall Response Rate (ORR; complete response [CR] +
partial response [PR], European Group for Blood and
Marrow Transplant [EBMT] criteria
1) to BZ monotherapy
in newly diagnosed, symptomatic MM
Secondary
­ Time to progression
­ Safety
­ Incidence and severity of PN
­ Impact of dose modifications on PN
­ Effect of pharmacologic interventions on PN
1. Bladé et al. Br J Haematol 1998;102:1115­23.

Patient Eligibility
Inclusion criteria:
­ Previously untreated, measurable MM
­ Nonsecretory or oligosecretory MM if measurable
­ No previous chemotherapy
­ Karnofsky Performance Status 60%
­ Platelets 50 x 10
9/L (or 30 x 109/L if extensive bone
marrow infiltration)
­ Adequate liver function
Exclusion criteria:
­ POEMS syndrome
­ Secondary malignancy requiring active treatment
­ HIV positive or active hepatitis
­ Grade > 2 PN
­ Steroids > 10mg daily of prednisone or equivalent

Study Design
Open-label, phase 2 study at 6 centers (USA)
BZ 1.3 mg/m
2 IV
Cycles 1­8
Long-term
follow-up
ologic
10-d rest
Neur evaluation
End of
Registration
D1
D4
D8
D11
treatment visit

Study Design
Tumor response assessed by EBMT criteria
1 every 2
cycles (6 wks)
­ Pts achieving CR received additional 2 cycles
beyond confirmation of CR
Pts with progressive disease (PD) or unacceptable
toxicity discontinued treatment
Candidates for SCT could proceed to SCT at
investigator's discretion
Planned treatment duration: up to 8 cycles
Dexamethasone- free approach
1. Bladé et al. Br J Haematol 1998;102:1115­23
.

Neurologic Assessments (All Sites):
Pts underwent neurologic examination by a
neurologist at screening, at the end of the study,
during therapy if screening results were abnormal,
or if clinically indicated (i.e. development of PN)
In addition, pts completed the Functional
Assessment of Cancer Therapy/Gynecologic
Oncology Group neurotoxicity (FACT/GOG-Ntx)
questionnaire on D 1 and 8 of each treatment cycle,
at end of study

Neurologic Assessments (DFCI):
Motor, sensory nerve conduction studies (NCS):
ˇ Sural NCS
ˇ Peroneal, posterior tibial motor NCS
ˇ Radial, ulnar sensory NCS + ulnar motor NCS
­ Quantitative sensory testing (QST) to assess small-fibers
­ Autonomic testing to assess small myelinated,
unmyelinated nerve fibers:
ˇ Quantitative sudomotor axon reflex testing (QSART)
ˇ Assessment of cardiovagal function by HR
variability to deep breathing, Valsalva maneuver
ˇ Assessment of sympathetic skin responses
ˇ QST to assess temperature, light touch,
vasodilation
Skin biopsies at screening, on development of PN, end of
treatment

Dose Modifications, Pharmacologic
Intervention for PN:
Bortezomib dose modifications for PN:
Severity of PN
Modification
G 1 without pain/loss of function
No action
G 1 with pain or G2
Reduce dose from 1.3 to 1.0
mg/m
2
G 2 with pain or G3
Withhold until PN resolves,
reinitiate at 0.7 mg/m
2 once wkly
G 4
Discontinue
Interventions for G 1 PN, neuropathic pain
­ Step 1: daily vitamins and/or nutritional supplements
­ Step 2: add gabapentin 300mg TID; titrate as tolerated up
to 1,200mg TID
­ Step 3: add nortryptiline 25mg qhs, increase up to 50mg
after 2 wks, then increase by 25mg monthly as tolerated
up to 100mg qhs; add duloxetine 20­60mg QD

Results: Pt Demographics,
Baseline Characteristics (N=66)
Median age, yrs (range)
60 (33­77)
Male, n (%)
45 (68)
Myeloma type, n (%)
IgG
28 (42)
IgG
8 (12)
IgA
7 (11)
Free light chain
2 (3)
Free light chain
2 (3)
light chain
1 (2)
Other/unknown
18 (27)
ISS myeloma stage, n (%)
I
21 (32)
II
14 (21)
III
31 (47)
Ig = immunoglobulin; ISS = International Staging System

Results: Pt Disposition
N (%)
Pts enrolled
66 (100)
Completed therapy (8 cycles)
30 (45)
Discontinued*
35 (52)
Evaluable for response
63 (95)
Evaluable for safety
65
(98)
Underwent neurophysiologic testing
34 (52)
*PD (9 pts), adverse events (8 pts, incl 2 for PN), CR (3 pts), other (14 pts)
One pt had screening visit only

Efficacy (N=63*)
Best Response to BZ treatment
n (%)
(EBMT)
CR
6 (10)
PR
19 (30)
ORR (CR + PR)
25 (40)
MR
14 (22)
CR + PR + MR
39 (62)
Stable disease (SD)
20 (32)
PD
4 (6)
*3 pts not evaluable

Time-to-event Analysis: DFCI
(n = 34)
32/34 (94%) responded (CR + PR + MR)
DOR (defined as time from best response to progression):
8.5 mos (range 1-19 mos)
14/32 responders progressed
18/34 (53%) progressed; 9 during treatment
Median TTP: 6 mos (range 1-24 mos)

Safety (n=54*)
Adverse Events (all grades)
n (%)
PN
38/65* (58)
Constipation
31 (57)
Nausea
26 (48)
Fatigue
23 (43)
Rash
18 (33)
Upper respiratory infection
9 (17)
Varicella zoster virus infection
3
(5)
*PN reported for all study centers: data collection for other adverse events
ongoing at one study center (RPCI)
All AE's were G 1­2 in severity except:
­ 1 G3 event: PN
­ 1 G4 event: fluid overload
­ 1 G5 event: bacterial meningitis

PN: Grade, Dose Modifications and
Reversibility
PN reported in 38/65 (58%) pts:
­ 24 G1
­ 13 G2
­ 1 G3
Dose reductions, discontinuations:
­ 4 pts with G1 reduction from 1.3 to 1.0 mg/m
2; 3 further
reduction to 0.7 mg/m
2
­ 9 pts with G2 reduction from 1.3 to 1.0 mg/m
2; 2 further
reduction to 0.7 mg/m
2
­ 1 pt with grade 3 PN discontinued
Preliminary analysis shows PN improved or
resolved in 6/8 pts (75%) with dose reduction

PN: Characteristics from
Neurophysiologic Testing (N=34)
n/N*
(%)
Baseline:
Small-fiber PN
at baseline
17/34 (50)
Large-fiber PN
at baseline
3/34 (9)
Post-treatment:**
New small-fiber PN

9/28 (32)
Worsened small-fiber PN

7/17 (41)
New large-fiber PN

4/25 (16)
*34 pts @ DFCI; 1 pt had screening visit only; some pts declined
post-treatment neurologic analysis
Small-fiber PN based on QST, autonomic studies, or both
Large-fiber PN based on abnormal sural sensory nerve action potential
**Preliminary Analysis (n=28)

Conclusions:
Single-agent BZ is active in pts with newly
diagnosed MM:
­ 10% CR
­ 40% ORR (CR + PR)
­ 22% MR
­ 6% PD
Toxicities manageable:
­ No unexpected AE's; no DVT's reported
­ Safety profile similar to previous studies
­ Varicella zoster virus infection noted prior to
prophylaxis with acyclovir, other anti-viral
therapy
­ "Steroid-sparing" approach

Conclusions:
Treatment-emergent PN (58%) manageable:
- lower grades noted (only 1 G3)
- reversibility c/w other studies
Underlying small-fiber PN appears more
common in MM than previously appreciated:
­ Evidence of small-fiber PN observed >50% of
pts with neurophysiologic testing at baseline
­ Small-fiber PN may emerge or worsen during BZ
treatment

Future Directions
Combination Therapy
Risk-adapted Approach
Optimal Sequence of Treatments
Further Evaluation of PN,
Characterization, Effect of Interventions

Acknowledgments: Study Teams
Dana-Farber Cancer Institute
Roswell Park Cancer Institute
Massachusetts General Hospital
Emory University
Memorial Sloan-Kettering Cancer Center
Beth Israel Deaconess Medical Center
Millennium Pharmaceuticals, Inc.
The Patients and their Families