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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

1

New Drug Approval: What

Does the Arrival of XPOVIO

(selinexor) Mean for Multiple

Myeloma Patients?

October 17, 2019

Tech Support

1-866-709-8255

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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

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Multiple Myeloma Research Foundation

Moderator

• Mary DeRome

Multiple Myeloma Research Foundation

Norwalk, Connecticut

www.themmrf.org

https://www.facebook.com/theMMRF

https://twitter.com/theMMRF

https://www.youtube.com/user/TheMMRF

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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

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Multiple Myeloma Research Foundation

Moderator

• Mary DeRome

Multiple Myeloma Research Foundation

Norwalk, Connecticut

www.themmrf.org

https://www.facebook.com/theMMRF

https://twitter.com/theMMRF

https://www.youtube.com/user/TheMMRF

Speakers

Ajai Chari, MD

Icahn School of Medicine at Mount Sinai

New York, New York

Paul G. Richardson, MD

Dana-Farber Cancer Institute

Boston, Massachusetts

Michael Walsh

Patient Advocate

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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

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Topics for Discussion

• Unmet needs for relapsing patients

• XPOVIO clinical data

• Patient management

• Future XPOVIO studies and use

• Patient perspective on XPOVIO

experience

Treatment Options for Relapsing

Patients

Paul G. Richardson, MD

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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

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Therapy for Relapsed/Refractory

Myeloma (RRMM): Numerous Choices

IMiDs

Proteasome

inhibitors

Chemotherapy

anthracyclines

Chemotherapy

alkylators Steroids

Novel

mechanisms

of action mAbs

Thalomid

(thalidomide)

Velcade

(bortezomib) Adriamycin Cytoxan

(cyclophosphamide)

Dexa- methasone

Farydak

(panobinostat)

Empliciti

(elotuzumab)

Revlimid

(lenalidomide)

Kyprolis

(carfilzomib)

Doxil (liposomal

doxorubicin) Bendamustine Prednisone Zolinza

(vorinostat)

Darzalex

(daratumumab)

Pomalyst

(pomalidomide)

Ninlaro

(ixazomib) Melphalan XPOVIO

(selinexor)

IMiD, immunomodulatory drug; HDAC, histone deacetylase; mAb, monoclonal antibody.

New formulations, new dosings, and new combinations

Despite Advances in Therapy, Multiple Myeloma Is Still

Considered Incurable; Almost All Patients Relapse

• Multiple myeloma is considered an

incurable, chronic disease

characterized by remissions and

relapses:

– Most patients experience multiple

relapses

– Some patients never respond, and

others stop responding to therapy

– Remission duration decreases with

each successive treatment regimen

– Patients with resistance to currently

available novel therapies have

particularly poor prognosis

18

13

7

5

0

5

10

15

20

1L 2L 3L 4L

Time to Progression,

mo Proportion Surviving

1.0

0.8

0.6

0.4

0.2

0.0

0 10 20 30 40 50

Months

Not triple-refractory (ie, double-refractory) (N=57)

Triple-refractory and

quad-refractory (N=148)

P Penta-refractory (N=70) =0.002

Line of Treatment

Harousseau JL et al. Blood. 2017;130:963; Sonneveld P et al. Haematologica. 2016;101:396; Rajkumar SV et al. Blood. 2011;117:4691-5;

Yong K et al. Br J Haematol. 2016;175:252; Gandhi UH et al. Leukemia. 2019;33:2266.

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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

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Median Overall Survival for Patients

Progressing After Darzalex

Pick M et al. Eur J Haematol. 2018;100:494.

Patients with heavily-pretreated

MM, receiving salvage therapy

post-Darzalex

100

80

60

40

20

0

0 6 12 18 24

Months

Percent Survival

DARATUMUMAB REFRACTORY

N: 22

Median OS: 3.5 months

Unmet Needs for RRMM Patients

Multiple myeloma remains largely incurable despite novel therapies, with RRMM

representing an area of exquisite unmet medical need.

A growing number of patients are exposed to proteasome inhibitors, immunomodulatory

drugs, and have become refractory to monoclonal antibodies, including Darzalex

(daratumumab) and Empliciti (elotuzumab).

Eventually, patients develop penta-exposed and triple-class–refractory* myeloma and

have a dismal prognosis.

Response rate and PFS progressively diminish with each relapse.

Until now, there were no approved drugs with established clinical activity in triple-class–

refractory myeloma.

*Refractory to proteasome inhibitors, immunomodulatory drugs, and CD-38 targeting mAbs, such as

Darzalex

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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

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Clinical trials

Relapsing After Multiple Prior Treatments

What options to consider?

ASCT, autologous stem cell transplantation; SCT, stem cell transplantation; BiTE, bispecific T-cell engager.

Commercial/

investigational

agents

• XPOVIO-based therapy

• 4-5 novel drug

combinations

• Multi-agent chemotherapy

and ASCT

• Allogeneic SCT (under

protocol-directed

conditions)

Investigational

agents

• CAR T-cell therapy

(eg, bb2121)

• Bispecific

antibodies/BiTEs (eg,

anti-BCMA)

• Venetoclax (in select

patients)

• Next-generation novel

agents, with novel

mechanisms of action

• Target-based approach

• Marizomib

Next- generation

proteasome

inhibitors

• Isatuximab

• Belantamab

mafodotin

• Idasanutlin

• MOR03087

• TAK079

• TAK573

• BiTEs

Monoclonal

antibodies

•Vemurafenib

• Afuresertib

• Dinaciclib

•Ibrutinib

•Trametinib

• Dabrafenib

• Ruxolitinib

Kinase

inhibitors

• Ricolinostat

HDAC

inhibitors

• Venetoclax

• Melflufen

Novel MOA

• Immune

checkpoint

inhibitors

̶ Pembro- lizumab

̶ Nivolumab

• Immune cell

therapy

̶ CAR-T

Immuno- therapies

• Iberdomide

(CC-220)

• CC-480

New

IMiDs

Selected New Agents

Under Investigation in Myeloma

IMiD, immunomodulatory drug; HDAC, histone deacetylase inhibitor; MOA mechanism of action;

BiTE, bi-specific T-cell engager; CAR T, chimeric antigen receptor T cells

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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

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Novel Mechanisms of Action in RRMM:

Key New Directions

How does XPOVIO (selinexor) work?

Without XPOVIO With XPOVIO

XPO1 in MM

• Transports >200 proteins from the nucleus to cytoplasm

• Expression increased in MM vs normal PC/MGUS/SMM

• Correlates with shorter survival and increased bone disease

Tai YT et al. Leukemia 2014;28:155; Schmidt J et al. Leukemia 2013;27:2357.

XPO1

selinexor

selinexor

XPOVIO (selinexor)

• Inhibits XPO1 through reversible covalent modification

• Nuclear retention/activation of tumor suppressor

proteins and glucocorticoid receptor

• Reduction of oncoproteins through nuclear

retention of their mRNAs

XPOVIO Clinical Efficacy, Safety,

and Patient Management

Ajai Chari, MD

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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

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Nuclear Export Inhibitor:

XPOVIO (selinexor)

Drug Formulation Approval

XPOVIO

(selinexor) Twice a week tablet

• In combination with dexamethasone for

relapsed/refractory myeloma patients who

have received at least four prior therapies

and whose disease is refractory to at least

two proteasome inhibitors, at least two

immunomodulatory agents, and an anti- CD38 monoclonal antibody

XPOVIO is from a new class of drug,

selective inhibitor of nuclear export (SINE)

Selinexor is a new agent for myeloma patients who

have exhausted many of the existing therapies.

XPOVIO (selinexor) Clinical Study

Design That Led to Approval

Oral XPOVIO Dexamethasone 80

mg

20 + mg

XPOVIO/dexamethasone twice weekly (days 1 and 3) – 28 day cycle

MM, multiple myeloma; PI, proteasome inhibitor; IMiD, immunomodulatory drug; ANC, absolute neutrophil count

Primary end point

• Overall response rate (ORR)

Secondary end points

• Duration of response (DOR)

• Clinical benefit rate (CBR)

• Overall survival (OS)

• Progression-free survival (PFS)

• Safety

Patient population

• MM previously treated with

Velcade, Kyprolis, Revlimid,

Pomalyst, Darzalex, an

alkylator, and glucocorticoids

• Disease documented to be

refractory to ≥1 PI, ≥1 IMiD,

daratumumab, a glucocorticoid

and last line of therapy

Key inclusion and

exclusion criteria

• Creatinine clearance ≥20 mL/min

• ANC ≥1,000/mm3

• Platelets ≥75,000/mm3

(if bone marrow plasma cell

>50%; platelets >50,000/mm3)

• Hemoglobin ≥8.5 g/dL

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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

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Prior Treatments of Patients

Enrolled Onto XPOVIO Clinical Trial

A total of 123 patients were enrolled, however 1 patient did not meet eligibility criteria, thus was excluded from this analysis.

IMiD, immunomodulatory drug; PI, proteasome inhibitor

N=122

Median prior regimens (range) 7 (3–18)

Number of prior treatment regimens

≤6

7–8

≥9

50 (41%)

36 (30%)

36 (30%)

Prior treatments

Refractory to PI/IMiD/daratumumab/glucocorticoid

Refractory to carfilzomib/pomalidomide/daratumumab

Refractory to 2 PIs/2 IMiDs/daratumumab

Stem cell transplant

≥2 Transplants

Intensive combination chemotherapy (eg, DT-PACE)

Daratumumab in last prior regimen

Daratumumab in combination

CAR T-cell therapy

122 (100%)

117 (96%)

83 (68%)

102 (84%)

29 (28%)

32 (26%)

58 (48%)

86 (70%)

2 (2%)

Efficacy of Selinexor in Relapsed/Refractory

Myeloma: XPOVIO + Dexamethasone

IMiD, immunomodulatory drug; PI, proteasome inhibitor

1. STORM Trial. Chari A et al. N Engl J Med. 2019;381:727; 2. Gavriatopoulou M et al. Presented at the 17th International Myeloma Workshop;

September 12–15, 2019. Abstract FP-110; 3. Vogl DT et al. Presented at the 17th International Myeloma Workshop; September 12–15, 2019. Abstract FP-111.

Additional analyses showed clinical benefit with

XPOVIO regardless of patient age and renal function.2,3

1.6 2.3 1.7 1.2

4.9 4.7 6.9 4.8

19.7 22.1

25.9

19.3

13.1

14.0

10.3

12.0

0

5

10

15

20

25

30

35

40

45

50

All Patients

(N=122)

Prior Dara in Combo

(N=86)

Prior Dara in Last Line

(N=58)

2 PI, 2 IMiD, &

Dara Refractory

(N=83)

sCR VGPR PR MR

ORR 26.2%

ORR 29.1% ORR 34.5%

ORR 25.3%

Percent Response

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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

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M protein changes shown for all patients with increases, and for patients with decreases ≤20% (30 patients with decreases 0–19% not shown).

CR, complete response; FLC, immunoglobulin-free light chain; MR, minimal response; PD, progressive disease; PR, partial response; SD, stable disease;

VGPR, very good partial response

• The majority of evaluable patients (71%)

had reductions in M protein

• ≥SD: 78.7%

XPOVIO Effect on M Protein

Overall Survival of Patients on XPOVIO in STORM

Compared to Historical FHAD Patients (Database)

N=64

Median OS 10.4 months

N=36

Median OS 5.8 months

FHAD, Flatiron Health Analytic Database

STORM Trial. Chari A et al. N Engl J Med. 2019;381:727.

STORM Patients

Overall Survival

FHAD Patients

Overall Survival

0 4 8 12 16

0

25

50

75

100

Months

Percent Survival

0 4 8 12 16

0

25

50

75

100

Months

Percent Survival

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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

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Adverse Events That Emerged

During XPOVIO Treatment

Event (% of patients) Grade 3 Grade 4 Total

Low platelets 25 33 73

Low RBCs/hemoglobin 43 1 67

Low neutrophils 18 3 40

Low leukocytes 14 0 33

Low lymphocytes 8 3 16

STORM Trial. Chari A et al. N Engl J Med. 2019;381:727.

Event (% of patients) Grade 3 Grade 4 Total

Fatigue 25 0 73

Nausea 10 0 72

Decreased appetite 5 0 56

Decreased weight 1 0 50

Diarrhea 7 0 46

Vomiting 3 0 38

Low sodium 21 1 37

Upper respiratory tract infection 2 0 23

Constipation 2 0 22

Difficulty breathing 4 0 22

Hematologic Toxicities Non-Hematologic Toxicities

Supportive Care Strategies at Mt. Sinai

• Ritalin 5–10 mg OD as needed

Gastrointestinal

Fatigue

• Platelets ≤50K or less: initiate N-Plate 10 mcg/kg weekly

Cytopenias

• Weekly CMPs

• IV fluids as needed with .9NS

• NaCl tabs 1GM TID

Low sodium

(hyponatremia)

• Bring patient back

in D3 or 4 of 1st

week of treatment

to assess for AEs

and provide IV

hydration as

needed

Na

Sodium

22.990

• Antiemetic Protocol:

− Zofran

− Rolapitant

− Olanzapine

• Most patients need a combination

of all 3 above antiemetics.

Reassess need after C1

• Contact your doctor if nausea, vomiting,

or diarrhea occur or persist

• Emend is avoided

− May potentiate the

activity with dex and

lead to hyperglycemia

• Avoid Megace (4 pts

developed clots)

• Maintain fluid intake

• Stay hydrated and active

• Report signs of bleeding right away

• Report signs of fatigue or shortness of breath

Patients Doctors

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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

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Selinexor and Backbone Treatments of MM

Patients (STOMP): Phase 1 Preliminary Results

STOMP Patient Characteristics SVd SPd SRd SDd SKd

Lines of therapy

≥1

Not V

refractory

≥2

including

PI and IMID

≥1

Not R25

refractory

≥3

including

PI and IMID

Patients enrolled 42 34 19 21 21

Median age, years 64 61 68 68 64

Median time diagnosis to treatment, years 5 6 4 5 4.5

Median prior regimens 3 4 1 4 4

Overall response rate 84% 50%* NR 74% 63%

ORR in backbone refractory 43% 38% NR N/A 67%

Progression-free survival 9.2 mos 10.3 mos NR NR 3.7 mos

PFS in backbone refractory 6.1 mos 4.8 mos NR N/A NR

*N=30 evaluable

Lonial S et al. NCCN 2018; Bahils et al 2018; Chen et al 2018; Jakubowiak et al ASH 2016; Gasparetto C et al Blood. 2018;132: Abstract 599;

White D et al. Presented at the 17th International Myeloma Workshop; September 12–15, 2019. Abstract OAB-083.

Summary

Due to aggressiveness of the disease and short half-life of drug, STORM treatment

begins with high doses of XPOVIO in an effort to obtain rapid disease control.

The XPOVIO clinical trial (STORM) included the largest, most heavily treated

patient population with MM in a prospective clinical trial to date.

XPOVIO plus dex treatment achieved an overall response rate of 26.2% in penta- exposed and triple class refractory MM.

Patient counseling, optimal supportive care, dose holds/modification associated with

less drug discontinuation due to AEs and increased RR, PFS, OS.

Accelerated approval demonstrates activity of XPOVIO/dex without additional anti- myeloma agents; however, given genomic complexity of RRMM, combination

strategies use only weekly XPOVIO and will likely be primary use.

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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

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Patient Perspective

Michael Walsh

• 59 years old

• Singer, actor, pianist

• Owner of home

recording studio in

Manhattan

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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

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Cancer Journey

• Diagnosed with lamda light chain multiple

myeloma on November 8th, 2013

• Treated with at least 11 lines of therapy including

3 bone marrow transplants

• Participated in 5 clinical trials that failed

• In 2017, from September to the end of the year,

spent 55 days in-patient trying to get my cancer

under control

XPOVIO (selinexor)

• In January, 2018 I took part in a clinical trial of

selinexor

• After just a few weeks taking selinexor, my light

chains dropped significantly from over 12,000

mg/l to under 800

• Took it again in May of 2019; light chains

numbers dropped from 19,000 to 300

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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

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Getting Hydration

Questions & Answers

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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

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Upcoming MMRF Webinar

For more information or to register, visit

theMMRF.org/PatientEd

Title Date Time

Maintenance Therapy November 2019 1:00 PM ET

American Society of Hematology (ASH)

2019 Meeting Highlights December 18, 2019 1:00 PM ET

MMRF Patient Summits

Saturday, November 2, 2019

Philadelphia, Pennsylvania

• Adam Cohen, MD—Co-Chair

University of Pennsylvania

• Edward Stadtmauer, MD—Co-Chair

University of Pennsylvania

Saturday, November 9, 2019

Toronto, Ontario, Canada

• Donna Reece, MD—Co-Chair

Princess Margaret Cancer Centre

• Suzanne Trudel, MD—Co-Chair

Princess Margaret Cancer Centre

For more information

or to register, visit

theMMRF.org/Patient

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New Drug Approval: What Does the Arrival of XPOVIO (selinexor) Mean for Multiple Myeloma Patients?

Webinar – October 17, 2019

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Right-Track Resources

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