Immunomodulation: The
Cornerstone of the Treatment
of Multiple Myeloma
Nikhil C. Munshi, MD
Associate Professor of Medicine
Harvard Medical
Medical School
School
Asso. Director, Jerome Lipper Myeloma Center
Dana-Farber Cancer Institute
Boston VA Healthcare System
DANA-FARBER
CANCER INSTITUTE

Faculty disclosure information
Consultant: Celgene, Millennium, Novartis
Grant/Research Support: No
Speakers Bureau: Celgene, Millennium,
Novartis
Major Stock Shareholder: No

Paradigm Change
From Targeting MM Cells
to Targeting BM Microenvironment
1960s - Targeting MM Cell
2005
- Targeting MM Cell
And BM Milieu
Melphalan, Prednisone,
Thalidomide, Bortezomib,
Radiation therapy
Lenalidomide, LPD, Hsp90
inhibitors, HDAC inhibitors
Hideshima T & Anderson KC Nat Rev Cancer 2002 2:927-937

Mechanism of Action of Thalidomide/IMiD
C.
C Cytokine
C
Cytokine effect
eeffect
MM cells
IL-6
TNF
B. Effect on
IL
IL--11
A.
A Direct
D
Direct effect
Adhesion
Bone marrow
Stromal cells
ICAM
ICAM--11
Bone marrow
Vessels
V
VEGF
IL
IL--22
bFGF
NK Cells
IFN
T Cell Co
Co--
D. Anti-angiogenic effect
eeffect
ti
s
l
mu t
a i
E. Immune
Immune effect
tion
E. Immune
Raje and Anderson N Engl J Med. 1999;341

RVD in patients with
relapsed/refractory MM
MM
Non-randomized, open-label, dose-comparison study
21-Day cycle (maximum 8 cycles)
1
4
8
11
14
21
B
B
B
B
Dex
Dex
Dex
Dex
Lenalidomide daily
daily
Lenalidomide 15 mg; bortezomib 1.0 mg/m
2; dexamethasone (on days 1, 2, 4, 5, 8, 9,
11, and 12) 40 mg in cycles 1­4, 20 mg in cycles 5­8
Per recent protocol amendment, starting dose of dexamethasone reduced to 20 mg in
cycles 1­4, with further reduction to 10 mg in cycles 5­8
Maintenance therapy consists of treatment with the study drugs at the dose levels
tolerated upon completion of cycle 8
RVD = lenalidomide + bortezomib + dexamethasone.
Richardson PG, et al. Blood. 2007;110 [abstract 2714].

RVD: efficacy
33 response-evaluable patients as of 15 Nov 2007
Bt
Best responses to d t
a e
­
nCR in 2 (6%)
­
PR in 16 (48%)
()
VGPR in 10 (30%)
­
MR in 6 (18%)
­
SD in 9 (27%)
(27%)
ORR (CR or nCR + PR + MR) 73%, including 55%
with CR or nCR or PR
Median duration of response in responding patients
is 7 cycles (range 4­16)
Median TTP, PFS, and OS have not yet been reached
Richardson PG, et al. Blood. 2007;110 [abstract 2714].

Phase I/II: VRDC EVOLUTION Study (N = 25)
Patients: 25 enrolled with previously untreated MM
Dose: RV
RVD with
with dose escalated
escalated CY 100 500mg/m
2
-500mg/m days 1 and 8;
pts could undergo SCT after 4 cycles
Best Unconfirmed Response:
ORR -
100%,
sCR -
20%
CR -
36%
VGPR -
68%
Stem Cell Collection: 11 pts collected a median of 5.9x106 CD34+
cells/kg; 2 pts required a second cycle of stem cell mobilization
Safety: Gr 3 AE 24% neutropenia 24%, PN 16%,
Thrombocytopenia 12%, no DVT
Kumar S et al. ASH 2008, abstract #93

Phase II Study: Sequential Bortezomib,
Cyclophosphamide, Dex (VCD) followed by
VTD
Patients: 44 enrolled with
with previously
previously untreated MM; median age 58
yrs (38-83); 72% ISS stage II/III
Response
VCD + VTD (n=43)
·
Estimated 12-mos OS and EFS
CR
26%
were 86% each after median
CR/nCR
35%
follow up
-
of 10 6
. mos
mos
VGPR
21%
PR
40%
·
22 pts proceeded to SCT; 100%
ORR
96%
had successful SC collection
Safety: 82% of pts complete 6 cycles; there were 11 Gr 3-4
events requiring
requiring dose adjustment
Bensinger W et al. ASH 2008, abstract #94

Overview of trials with lenalidomide
combinations in newly diagnosed MM
CR or nCR,
TTP or PFS,
Md
Me i
dian OS
OS,
Regimen
ORR, %
VGPR, %
%
months
months
Not reached
Not reached
RVD
1
RVD
100
44
74
(> 8 mos)
(> 8 mos)
VDCR
2
100
36
68
Not reached
Not reached
Not reached
Not reached
RCD
3
85
NR
32
(> 2 yrs)
(> 2 yrs)
MPR
4
81
24
48
29
91% @ 2 yrs
1. Richardson PG, et al. Blood 2008;112:[abstract 1742]. 2. Kumar S, et al. Blood
2008;112[abstract 93]. 3. Kumar S, et al. Blood 2008;112:[abstract 91]. 4. Palumbo
A, et al. Blood 2008;112:[abstract 2768].


RAD in relapsed MM
Day
1
2
3
4
17 18 19 20 21
28
Lenalidomide, p.o.
Doxorubicin, 24-h continuous i.v.
Dexamethasone, p.o.
Patients,
Lenalidomide,
Doxorubicin,
Dexamethasone,
Pegfilgrastim
Dose level
n
mg/day
mg/m
2
mg/day
1
3
10
4
40
­
2
3
10
6
40
­
3
3
10
9
40
­
4
6
15
9
40
­
4 + G-CSF
3
15
9
40
6 mg, day 6
5+G
5 + -
G CSF
6
25
9
40
6 mg
mg, day 6
RAD = lenalidomide + doxorubicin + dexamethasone.
Knop S, et al. Blood. 2007;110 [abstract 2716].

RAD trial: efficacy
Response other than nCR or CR
100
nCR
87
CR
80
)
60
60
(%
60
tsn
40
Patie
20
25
23
0
Dose levels 1­4
Dose level 5
(n = 20)
+ G-CSF
(n = 30)
Cytogenetic abnormality
Patients, n/evaluable (%)
PR, n
del(13q)
17/37 (46)
12
t(4;14)
4/25 (16)
3
del(17p)
6/31 (19)
2
Knop S, et al. Blood. 2007;110 [abstract 2716].

Overview of trials with lenalidomide
combinations in relapsed/refractory MM
CR or nCR,
TTP or PFS,
Median OS,
Regimen
g
ORR, %
,%
VGPR, %
,%
%
th
mon smo th
n s
Len + Dex
1
61
24
NE
11
35
8
Len + Dex
2
Dex
60
25
NE
11
RMPT
3
76
13
33
55% @ 1 yr
66% @ 1 yr
RAD
4
RAD
73
15
45
45
88% @ 1 yr
RVD
5
78
20
29
Not reached
Not reached
6
RCD
65
5
20
Not reported
Not reported
DVd-R
7
75
29
NE
12
Not reached
1. Weber DM, et al. N Engl J Med. 2007;357:2133-42. 2. Dimopoulos M, et al. N Engl J Med. 2007;357:2123-32. 3. Palumbo
A, et al. Blood 2008;112:[abstract 868]. 4. Gerecke C, et al. Blood 2008;112:[abstract 2782]. 5. Anderson KC, et al. J Clin
Oncol 2008;26:[abstract 8545]. 6. Morgan GJ, et al. Br J Haematol. 2007;137:268-9. 7. Baz R, et al. Ann Oncol.
2006;17:1766-71. 8. Weber D, et al. Blood 2007;110;[abstract 412].

CALGB-100104: lenalidomide as maintenance
therapy after autologous PBSCT
Ongoing phase III, randomized, placebo-controlled trial
Patients with active MM, stable disease, or disease responsive to 4 months'
induction therapy (N = 588)
PBSCT
Re-staging
90­100 days after PBSCT
Randomize
Lenalidomide
Placebo
10
/d
mg ay
/d
p.o.
10
/d
mg ay
/d
p.o.
(n = 250)
(n = 250)
Primary end-
end point: time to disease progression
progression after autologous PBSCT
Secondary end-points: CR rate, PFS, OS, feasibility of long-term lenalidomide
PBSCT = peripheral blood stem cell transplant.
www.clinicaltrials.gov.

Rationally based combination therapies
·
Lenalidomide and anti CD40
-
antibody
·
Lenalidomide and mTOR inhibitor
·
Lenalidomide and doxil
·
Lenalidomide and HuLuc63
·
Lenalidomide and LBH 589
·
Lenalidomide and perifosine
p
·
Lenalidomide and bevacizumab
·
Lenalidomide and vaccine

Phase I trial of perifosine, Len, and
Dex in
in relapsed/refractory
relapsed/refractory MM
Day
1 2 3 4
9 10 11
11 12
17 18 19 20 21
28
Perifosine 50 or 100 mg/day
Lenalidomide 15 or 25 mg/day
D
D
D
Cycles 1­4 only
D
Cycles 5+
D = dexamethasone 20 mg
Results
·
32 patients enrolled, median age 61 (range 37­80) years
·
Median 2 (range 1­4) prior therapies, including Dex (94%), Thal (83%), Bort (47%)
·
ORR ( PR) 50%, VGPR 17% (VGPR 10%; nCR
nCR = 7%)
7%)
·
Median TTP for patients having PR is 31 (range 11­79) weeks
·
Grade 3 or 4 adverse events include neutropenia (20%), hypophosphataemia
(17%), thrombocytopenia (13%), anaemia (10%), fatigue (79%)
Bort = bortezomib; nCR = near-complete response;
Jakubowiak A, et al.
ORR = overall response rate; PR = partial response;
Blood. 2008;112:[abstract 3691].
TTP = time to progression; VGPR = very good PR.

NPI-0052 + Lenalidomide Trigger
Synergistic/Additive Anti-MM Activity
Synergistic
Additive
MM.1S cells
MM.1R cells
CI = 1 0
. 0
00
CI < 1.00
CI = 1.00
CI < 1.00
O hr
24 hr
48 hr
Rev
NPI-0052
Dharminder Chauhan et al., 2009

NPI-0052 + Lenalidomide Trigger
Synergistic/Additive Anti-MM Activity in Patient cells
Dharminder Chauhan et al., 2009

Combination of lenalidomide and NPI-0052
Prolongs Survival
Oral NPI-0052 (X2 WK) and lenalidomide (X4 WK)
Pv
P al
va ue
u 0
-. 01
001
110
100
Control
90
Rev 5 mg + NPI 0.15 mg/kg
NPI-.150mg/kg
eic
80
Rev-2.5mg/Kg
M
70
Rev 2. 5 mg
mg + NPI 0 15
.
mg/kg
70
e
Rev 5mg/Kg
60
liv
50
Rev 2.5 +NPI-.150mg/Kg
Af
40
Rev 5+ NPI-.150mg/Kg
o
30
mg/kg
%
20
0.15
10
NPI
0 0 25 50 75 100 125 150
Survival (Days)
1- Animals in control, Rev 2.5 and 5 mg died between 30-45 days.
2- Low dose NPI-treated animals have 10-15 days more survival.
3- Animals in combination group
gp survived 4-5 month.
Dharminder Chauhan et al., 2009

IMiDs Augment anti-MM Immunity
T Cell
NK Cell
NFA
NF T
A
IL-2
IL-2
PKC
D
d
en ritic Cell
PI3K
CD28
Apoptotic
MM Cell
MM Cell
IMiDs
VEGF
IL-6
Bone Marrow Stromal Cell
LeBlanc R et al. in press Hayashi T et al. in press

IMiD3 Overcomes CTLA-
CTLA 4 Blockade
Blockade
100000
80000
60000
pmc
40000
20000
0
T-cells Imm Mat
DCs DCs
antiCD3
Immature DCs
mature DCs
T
ll
-ce s+IMiD
3
IMiD
T-cells+CTLA-4-I
g

Treatment of PBMCs with Thal and IMiD
increases lysis
y
of MM cell lines
DMSO
Thal
1ug/ml
40
30
5ug/ml
20
lysis 20
15
ysis
cell 10
l
%
10
0
cell
5
30:115:17.5:1
%
0
effector:target ratio
30:1
15:1
75
7.5:1
effector:target ratio
IMiD1
DMSO
40
Thal
s
IMiD1
30
IMiD2
lysi 20
IMiD3
cell 10
%
0
30:1
15:1
7.5:1
effector:target ratio

IMiDs Enhance NK Cytotoxicity against MM Cells
Natural cytotoxicity
ADCC
DMSO
DMSO
Target: U266
Target: MM.1S
IMiD1
40%
IMiD1
40%
IMiD3
IMiD3
30%
city 30%
ity
20%
totoxi 20%
totoxic
Cy
Cy
10%
10%
0%
0%
40:1
20:1
10:1
E : T 60:1
30:1
60:1
30:1
Effector : Target
Control Ab
rhuCD40 mAb
Target: RPMI8266
Target: ARH-77
20%
40%
30%
c
city
city 15%
10%
20%
totoxi
totoxi
Cy
Cy
5%
10%
0%
0%
40 : 1
20 : 1
10 : 1
E : T 60:1
30:1
60:1
30:1
Effector : Target
Hayashi T et al. in press
Control Ab
rhuCD20 mAb

Lenalidomide Induces
Induces Increased anti
anti--CD40
CD40
induced ADCC Against Autologous MM Cells
Patient 1
Patient 2
Patient 3
60
**
50
**
25
** **
50
40
**
20
lysis
40
30
15
fic
30
*
20
10
*
10
20
*
*
10
5
Speci
10
%
0
0
0
L
L
L
on
G
o
40
de
+
on
G
40
de
+
on
G
o
40
de
4
+
c
Ig
S
c
Ig
S
c
Ig
S
med
con
SGN-
med
con
SGN-
med
con
SGN-
lenalidomi
lenalidomi
lenalidomi
Clinical Trial in MM in 2008
Lenalidomide + SGN40
Tai et al. Cancer Res 2005, 65: 7896-7901.
Lenalidomide + anti-CS-1

Immunomodulatory role of lenalidomide
on pneumococcal responses
responses
Cohort A
Lenalidomide 25 mg, days 1-21, every 28 days,
6 cycles
Pneumococcal vaccine day 0 cyc
cy le
cle 1 and
and day
Relapsed MM
15 cycle 2
patients,
lenalidomide naïve,
treated with 3 prior
therapies
p
Cohort B
Lenalidomide 25 mg, days 1-21, every 28 days,
6 cycles
Pneumococcal vaccine day 15, on cycles 2
and 4
Noonan KA, et al. Blood 2008;112:[abstract 2772].

Immunomodulatory role of lenalidomide
on pneumococcal responses:
responses: results
Cohort A:
A: 80% decrease in
in antibody titres
titres, 0%
0% of
of total
total T
cell population proliferated to CRM-197
Cohort B: 2 fold increase in antibody responses, 1.8% of
total T cell population proliferated to CRM-197
·
Antibody responses more pronounced in BM ers
v
s
u PBL
·
MCP-1 and MIP-1 levels decreased during trial
·
IFN and IL-
IL 17 undetectable in
in PBL but were elevated
and unchanged respectively in BM samples
Noonan KA, et al. Blood 2008;112:[abstract 2772].

Randomized Study to evaluate ability of
Lenalidomide to
to Augment
Augment Vaccine
Vaccine Response
VA
V /DFCI
A
Randomized Study
Hepatitis B
Day 28
Vaccine
V
Arm I. Lenalidomide
AI
Arm I. L
lid
ena
id
om e
25 mg/day PO, Days 11­
­77
25 mg/day PO, Days 88­
­14
14
Patients with
Anti-HepB
MM
Immune
response
Arm II. Placebo
Arm II. Placebo
Days 1-7
Days 8-14

Pomalidomide in Myeloma
C
MM cells
IL-
IL 6
TNF
B
IL-1
A
BM
Bone Marrow
ICAM-1
Stromal Cells
Bone Marrow
Vessels
NFAT
PKC
IL-2
IL-2
IFN
NK Cells
VEGF
PI3K
CD28
bFGF
CD8+ T
Dendritic
Cells
D
E
Cells
Mitsiades et al. Blood 99: 4525, 2002
Hideshima et al. Blood 96: 2943, 2000
Lentzsch et al Cancer Res 62: 2300, 2002
Davies et al. Blood 98: 210, 2001
LeBlanc R et al. Blood 103: 1787, 2004
Gupta et al. Leukemia 15: 1950, 2001
Hayashi T et al. Brit J Hematol 128: 192, 2005

In Vivo Anti-Tumor Activity of Thalidomide/IMiDs
)
3 B
A
100
(mm
3000
80
rvival
60
u
lume
2000
S
o
40
%
rv
1000
20
om
0
0
Tu
0
25
50
75
100
125
0
7
14
21
28
Days of treatment
Days of treatment
C
50
45
40
Thalidomide
35
IMiD1
30
25
IMiD3
20
15
10
5
0
Contro
IMiD1
IMiD3 Thalidomide
l
Treatment
Lentsch et al. Leukemia 2003:17:41

Phase II trial of pomalidomide + low-
dose Dex
Dex in relapsed/refractory MM
MM
Day
1
8
15
22
28
Pomalidomide 2 mg/day
D
D
D
D
D = dexamethasone 40 mg/day
Aspirin 325 mg/day as routine thromboprophylaxis
Results
·
37 patients enrolled, median age 66 (range 40­88) years
·
3 prior regimens (38%), 2 prior regimens (35%), 1 prior regimen (27%),
prior ASCT (76%)
(76%)
·
ORR ( PR) 62%, VGPR 24%
·
Adverse events in 5% of patients
­ grade 3 neutropenia (31%), thrombocytopenia (3%), anaemia (3%)
ASCT = autologous stem cell transplant; ORR = overall
response rate; PR = partial response; VGPR = very good PR.
Lacy MQ, et al. Blood. 2008;112:[abstract 866].

Phase II trial of Pomalidomide in
Relapsed/Refractory
py Myeloma
y
(N
( = 60)
·
Dose: Pomalidomide - 2mg po daily days 1-28
Dexamethasone - 40mg po
po days 1, 8, 15
15 & 22
Aspirin - 325mg po days 1-28
Confirmed Response
Response
N=
N 60
=60
sCR
1 (2%)
CR +VGPR 25%
VGPR
14 (23%)
ORR 58%
58%
PR
20 (33%)
SD
11 (18%)
Among the first
first 37 patients, there were
were 13
13 lenalidomide
lenalidomide refractory
refractory
patients, with responses seen in 29%
One death due to pneumonia while neutropenic
Primarily fatigue Grade ¾
28%
Neutropenia
32%
No DVT
Lacy et al, ASH 2008; Abstr. 866

Nearing The Cure
Improving OS from the Time
Impact of novel agents on
of Relapse Post-ASCT with
outcome in newly diagnosed
Novel Agents (n=387)
disease (n=2981)
70
1.0
Relapsed before 1998
60
Relapsed 2000­2001
0.8
Relapsed 2004­2005
50
al
(months)
viv 0.6
S 40
v
O
Sur
30
0.4
Median 20
P < 0.001
0.2
10
Multi Agent Sequential
gq
Therapy
py
0.0
Improves Sustained CR
1971­ 1977­
1983­
1989­ 1995­
2000­
0
20
40
60
80
100
1976
1982
1988
1994
2000
2006
­ Is it a Cure? (n=2981)
Year of diagnosis
Kumar
100%
et al. Blood 2008;111: 2516Z
TT3
(13/162)
CR DURATION
Kumar et al. Blood 2007;110: (#3594)
80%
P=0.0008
TT2+Thal
60%
(78/201)
P=0.19
40%
TT2-Thal
(67/148)
20%
P=0.0002
TT1
(78/94)
0% 0
5
10
15
20
Years from complete response
Barlogie et al. ASCO 2008, #8516

Thank you