the spectrum of mds: disease management and patient education
TRANSCRIPT
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The Spectrum of MDS: Disease Management and
Patient Education
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Disclaimer
A presentation timeslot has been assigned to provide a Symposium supported by the Aplastic Anemia and MDS International Foundation and Medical Learning Institute during the Oncology Nursing Society’s (ONS) Congress Virtual Event. The Oncology Nursing Society's assignment of a presentation timeslot does not imply product endorsement.
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Faculty
Catherine Chittick, RN, BSNOncology Nurse
Massachusetts General Hospital Cancer CenterBoston, MA
Jenna Moran, MSN, FNP-BCOncology Nurse Practitioner
Massachusetts General Hospital Cancer CenterBoston, MA
Aura Ramos, RN, BSNLeukemia Research Nurse
Massachusetts General Hospital Cancer CenterBoston, MA
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Learning Objectives
After completing this activity, the participant should be better able to:
• Explain the methods used to diagnose, stratify and treat MDS; • Identify current and emerging treatment options for patients with
low-risk MDS, high-risk MDS, and secondary AML;
• Analyze the nurse's role in managing treatment for MDS based on clinical presentations, diagnostic workups, transfusion dependence, comorbidities, and clinical trial consideration;
• Discuss strategies to address treatment goals and decision-making, educating patients and provide palliative care to improve outcomes.
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Meeting Agenda
8:30 - 8:35 am Introduction
8:35 - 9:15 am MDS Diagnosis and Treatment Update - molecular testing, emerging therapies for chronic anemia, stem cell transplantation options, and combination therapies
9:15 - 9:35 am Disease Management – helping patients manage side effects of treatment, including RBC transfusion dependence and fatigue, treatment adherence and clinical trial participation
9:35 – 9:50 am Quality of life issues, including goals of care conversations and psychosocial aspects of living with MDS
9:50 – 10:00 am Question & Answer Session
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CE Information• Providership, Credit and Support
This accredited continuing education (CE) activity is jointly provided by Medical Learning Institute, Inc. and the Aplastic Anemia and MDS International Foundation.
This accredited CE is supported by an independent educational grant from Bristol Myers Squibb and Gilead Sciences Inc.
• Target AudienceThis activity is intended for nurses and nurse practitioners engaged in the care of patients with Myelodysplastic Syndromes (MDS).
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Credit Designation
Nursing Continuing Professional Development (NCPD) Medical Learning Institute, Inc. is accredited as a provider of nursing continuing professional development by the American Nurses Credentialing Center’s Commission on Accreditation.Successful completion of this nursing continuing professional developmentactivity will be awarded 1.5 contact hours and 1.12 contact hours in the area of pharmacology
Nurse Practitioner Credit Designation (AANP) This activity has been submitted to the American Association of Nurse Practitioners for approval of up to 1.5 contact hours of accredited education. .
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Faculty DisclosuresCatherine Chittick, RN, BSN• Has no disclosures
Alice Houk, MPS• Has no disclosures
Jenna Moran, MSN, FNP-BC• Has no disclosures
Aura Ramos, RN, BSN• Has no disclosures
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DisclosureBefore the activity, all faculty and anyone who is in a position to have control over the content of this activity and their spouse/life partner will disclose the existence of any financial interest and/or relationship(s) they might have with any commercial interest producing healthcare goods/services to be discussed during their presentation(s): honoraria, expenses, grants, consulting roles, speakers bureau membership, stock ownership, or other special relationships. Presenters will inform participants of any off-label discussions. All identified conflicts of interest are thoroughly vetted by Medical Learning Institute ,Inc. for fair balance, scientific objectivity of studies mentioned in the materials or used as the basis forcontent, and appropriateness of patient care recommendations.
The associates of Medical Learning Institute ,Inc., the accredited provider for this activity and Aplastic Anemia and MDS International Foundation, do not have any financial relationships or relationships to products or devices with any commercial interest related to the content of this CE activity during the past 12 months.
Name of Manager/Planner Title Reported Financial Relationship
Bobbie Perrin, RN, OCN Content Expert Reviewer Has nothing to disclose.
Tracy L. Greene, MSN, RN, NP-C Lead Nurse Planner Has nothing to disclose.
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Instructions for Credit
There are no fees for participating in or receiving credit for this accredited activity. To obtain CEUs for this program, participants must complete the post-test and evaluation form. A score of 70% or higher is needed to obtain nurse credit. Please submit the online evaluation at the link provided in an e-mail sent to registered attendees after the program. The CEU certificate will be sent via e-mail within two business days of the evaluation submission. For questions regarding the accreditation of this activity, please contact Medical Learning Institute, Inc. at (609) 333-1693 or [email protected].
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Access to program slides and AAMDSIF materials:
https://www.aamds.org/ons-2021
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OVERVIEW OF MDS• Myelodysplastic syndrome comes from the Greek origin
• “myelo” prefix means marrow • “dysplasia” means abnormal looking cells that do not function properly
• Myelodysplastic syndromes (MDS) are cancers of the hematopoietic progenitors characterized by three major components
1. Bone marrow failure2. Clonal expansion 3. Risk of progression to acute leukemia
• MDS shares clinical and pathogenic features with acute myeloid leukemia (AML) but MDS has a lower percentage of blasts in peripheral blood and bone marrow (<20%) at the time of diagnosis
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EPIDEMIOLOGY
MDS occurs more commonly in older adults
• The median age at presentation is 70 years
• Disease onset before the age of 50 is unusual
• Exception: therapy-related MDS
Am J Med. 2012 Jul; 125(7 Suppl): S2–S5.
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EPIDEMIOLOGY
• Incidence: 10,000 cases per year in the US
• Risk factors: • Exposures leading to bone marrow injury
• cytotoxic chemotherapy• ionizing radiation
• Environmental exposures • Tobacco• benzene
• Inherited genetic conditions (rare)• Down Syndrome, Diamond Blackfan anemia, Fanconi anemia, Li-Fraumeni syndrome
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MEDIAN SURVIVAL
• Median survival is widely variable ranging from <1 year to > 10 years
• Cause of death is usually from consequences of bone marrow failure or progression to AML
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PATHOPHYSIOLOGY
• MDS is defined by three key features:
• Clonality
• Dysplasia
• Cytopenias
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PATHOPHYSIOLOGY• Clonality: a population of blood or bone marrow cells that share
an acquired mutation
• Most often detected by next generation sequencing of DNA (molecular panel) or by cytogenetics (karyotype) or FISH
• Cytogenetic mutations: recurrent chromosomal abnormalities• ~50% of patients • Del(5q), del(7q), t(11;16)(q23.3;p13.3)
• Molecular mutations: single gene mutations typically identified through next generation sequencing of the DNA
• ~90% of patients• Early disease: TET2, IDH1, IDH2, DNMT3A and ASXL1• RNA splicing mutations: SF3B1, SRSF2• Late disease: JAK2, RUNX1, KRAS, NRAS and TP53
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PATHOPHYSIOLOGY
• Dysplasia: abnormal shape and appearance of cells under the microscope. These cells are unable to function properly
dysplastic neutrophils dysplastic erythrocytes
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PATHOPHYSIOLOGY
• Cytopenias: a reduction in the number of mature blood cells (red blood cells, white blood cells and platelets)
• Hgb <11 g/dL
• ANC < 1500
• Platelets <100 x 10^9/ L
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CLINICAL PRESENTATION
• Common presenting signs/ symptoms include:
• Bleeding/ bruising• Fatigue• Shortness of breath• Infections• Routine CBC detects low blood counts
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WORKUP• History and Physical
• Details of cytopenias (fatigue, infections, bruising/ bleeding)• Evaluate for other causes: nutrition, alcohol, medications, toxic exposures, prior treatment
with antineoplastic agents)• Ask about siblings if suspect high-risk MDS
• Labs• CBC with differential = most important
• Red Blood Cells: anemia with low retic count• White Blood Cells: approximately half of patients have a reduced total white blood cell
count with blasts <20% on differential• Platelets: approximately one quarter of patients have thrombocytopenia
• Reticulocyte count• Erythropoietin level• Copper and ceruloplasm• B12, folate
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DIAGNOSTICS
• Blood Smear: • Assesses the size, shape and
general appearance of cells• In MDS will demonstrate
dysplasia in the red and white blood cells and sometimes platelet abnormalities
• Bone Marrow Biopsy: • Gold standard for the
diagnosis and classification of MDS
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BONE MARROW BIOPSY• Morphology: how cells appear when evaluated under the microscope
• Cytochemistry and immunochemistry: iron stain required to detect ringed sideroblasts; other stains may also be helpful to identify the cellular lineage
• Flow Cytometry: multiparameter flow may be helpful in assessing diagnostic and prognostic features of MDS
• Cytogenetics: patients may have single or multiple chromosomal changes at the time of diagnosis or chromosomal changes may appear during the course of the disease
• Most common abnormalities: complex karyotype, del(5q), trisomy 8 and del(20q)
• Molecular Diagnostics: mutations found in genes that become driver mutations • Detected on next generation sequencing (NGS)
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CASE STUDY: MR. F
Mr. F: presented 2016 without symptoms to PCP for routine blood work. CBC revealed mild anemia and mild leukopenia.
CBC with differential: WBC: 3.72HGB: 8.2HCT: 24.3PLTS: 338ANC: 2700
Review of Systems notable for: fatigue, occasional hemorrhoidal bleeding
He was referred to hematology for a bone marrow biopsy.
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CASE STUDY: MRS. L
Mrs. L: presented 2021 with 2-week history of shortness of breath, fatigue and chest palpitations on exertion. She was initially concerned for Covid-19 infection and presented to urgent care clinic. Covid-19 testing was negative. She was diagnosed with a possible sinus infection and prescribed a course of amoxicillin. Her symptoms did not improve, and she presented to her PCP two weeks later. A CBC revealed pancytopenia with 9.6% peripheral blasts. Peripheral blood smear showed dysplasia and a single auer rod.
CBC with differential: WBC: 1.91HGB: 6.3HCT: 18.9PLTS: 15ANC: 400Blasts: 9.6%
She was admitted for further workup of suspected leukemia/ myelodysplastic syndrome
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CLASSIFICATION OF MDS: WHO 2016 CRITERIA
• Myelodysplastic syndromes (MDS) are classified using the World Health Organization (WHO) classification system
• Most recently updated in 2016
• Divides MDS into six different types based on morphology, dysplasia, cytopenias, amount of ringed sideroblasts (early red blood cells), blasts, and chromosomal changes
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2016 WHO MDS CLASSIFICATION
WHO 2008 WHO 2016 Characteristics
RCUD(RA/RN/RT)
MDS w/ single lineage dysplasia (MDS-SLD) 1 dysplastic lineage, 1–2 cytopenias, <15% RS*, <5% BM/<1% PB blasts, no Auer rods
RCMD MDS w/ multilineage dysplasia (MDS-MLD) 2–3 dysplastic lineages, 1–3 cytopenias, <15% RS*, <5% BM/<1% PB blasts, no Auer rods
RARS MDS w/ ring sideroblasts (MDS-RS) ≥15% RS or ≥5% RS if SF3B1 mut present, <5% BM/<1% PB blasts, no Auer rods
Del(5q) MDS w/ isolated del(5q) Del(5q) alone or w/ 1 abnl except –7 or del(7q)
RAEB-1RAEB-2
MDS w/ excess blasts (MDS-EB) EB-1: 5–9% BM/2–4% PB blasts, no Auer rodsEB-2: 10–19% BM/5–19% PB blasts or Auer rods
MDS-U MDS, unclassifiable (MDS-U) 1% PB blasts, single lineage dysplasia & pancytopenia,or defining cytogenetic alteration
Arber DA et al. Blood 2016;127:2391
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R-IPSS SCORING SYSTEM
• Revised International Prognostic Scoring System (R-IPSS)
• Predicts both risk and median survival
• Guides when and how to treat a patient
• Score based on: • Bone marrow blasts (%)• Cytopenia• Cytogenetics• Age
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R-IPSS SCORING SYSTEM (2012)
Prognosticvariable
0 0.5 1 1.5 2 3 4
Cytogenetics Very good - Good - Intermediate Poor Very poor
Bone marrowblast (%)
</= 2 - >2% - <5% - 5-10% >10% -
Hemoglobin >/= 10 - 8 - <10 <8 - - -
Platelets >/= 100 50-<100 >50 - - - -
ANC >/= 0.8 < 0.8 - - - - -
- indicates not applicable
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R-IPSS SCORING SYSTEM (2012)
Characteristics
Cytogenetic Group
Very good -Y, del(11q)
Good Normal, del(5q), del(12p), del(20q), del(5q) + 1 additional abnormality (other than del7)
Intermediate Del(7q), +8, +19, I(17q), other abnormalities not in other groups
Poor -7, inv(3)/t(3q),-7/del(7q) + 1 additional abnormality, complex (3 abnormalities)
Very Poor Complex (>3 abnormalities)
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R-IPSS SCORING SYSTEM (2012)RISK CATERGORY RISK SCORE
Very Low <= 1.5
Low > 1.5 – 3
Intermediate >3 – 4.5
High > 4.5 – 6
Very High > 6
Number of patients
Very low Low Intermediate High Very High
Patients (%) 7012 19% 38% 20% 13% 10%
Survival 8.8 5.3 3.0 1.6 0.8
AML/ 25% NR 10.8 3.2 1.4 0.7
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R-IPSS SCORING SYSTEM (2012)RISK CATERGORY RISK SCORE
Very Low <= 1.5
Low > 1.5 – 3
Intermediate >3 – 4.5
High > 4.5 – 6
Very High > 6
Number of patients Very low Low Intermediate High Very High
Patients (%) 7012 19% 38% 20% 13% 10%
Survival 8.8 5.3 3.0 1.6 0.8
AML/ 25% NR 10.8 3.2 1.4 0.7
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R-IPSS SCORING SYSTEM (2012)
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Greenberg et al, Blood 2012
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POLLING QUESTION:
Which statement best describes the importance of next generation sequencing?
A)Identifies molecular mutations specific to the patient’s cancer B) Identifies potential targeted therapy treatment options C) Provides no information regarding response to treatmentD)Both A+B
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POLLING QUESTION:
What mutation would you consider to be one of highest risk/associated with a poor prognosis?
A) NPM1 mutationB) TET2 mutationC) SF3B1 mutationD) TP53 mutation
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CASE STUDY: MR. F
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CASE STUDY: MRS. L
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POLLING QUESTION:
The R-IPSS scoring system considers which of the following in determining the patient’s risk stratification:
A) Blast % in the bone marrow at the time of diagnosisB) PLT count at the time of diagnosisC) Molecular data (P53 mutation)D) Both A+B
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MDS TREATMENT
• Goal of therapy: • Control symptoms• Improve quality of life• Improve overall survival• Prevent progression to acute myeloid
leukemia
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MDS TREATMENTApproved and Emerging Therapies
• Low risk MDS treatment options:• Supportive care
• Red Blood Cell and Platelet transfusions• Red Cell Growth Factors
• ESA: epoetin (Procrit and Epogen) and darbepoetin (Aranesp)• Lenalidomide (Revlimid)• Luspatercept
• High Risk MDS treatment options: • Hypomethylating Agents:
• azacitidine and decitabine • Oral decitabine• Venetoclax (approved for AML but also used in MDS)• IDH inhibitors (approved for AML but also used in MDS)• Stem Cell Transplant
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MDS TREATMENT Lenalidomide (Revlimid):
FDA approved in 2005 for patients with low- or intermediate-1 risk myelodysplastic syndromes with a 5q- syndrome but no other additional cytogenetic abnormalities
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MDS TREATMENT Erythropoietin Stimulating Agents (ESAs):
• Darbepoetin (Aranesp) • Approved by the FDA in 2001 for anemia caused by cancer• Start with lowest dosing possible to avoid red blood cell transfusions • Serum epo </= 500• Assess response after 3 months
• Epoetin (Procrit/ Epogen)• Approved by the FDA in 2008 for anemia caused by cancer• Serum epo </= 500
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MDS TREATMENT Luspatercept (Reblozyl)
• Approved in 2020 for anemia due to myelodysplastic syndrome
• Treatment of anemia requiring 2 or more red blood cell transfusions over 8 weeks in adults with very low to intermediate risk MDS-RS
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MDS TREATMENT Hypomethylating Agents
• Azacitidine (Vidaza)• FDA approval in 2004 for MDS with the following French-American British (FAB)
classification subtypes: refractory anemia or refractory anemia with ringed sideroblasts (transfusion dependency), refractory anemia with excess-blasts in transformation and CMML
• Administered IV or SC x 7 days in a 28-day cycle
• Decitabine (Dacogen)• FDA approved in 2006 for MDS with FAC subtypes of RA, RARS, RAEB, RAEB-T and
CMML as well as intermediate-1, intermediate-2 and high-risk R-IPSS • Administered IV x 5 days in a 28-day cycle
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MDS TREATMENT Hypomethylating Agents
• Oral decitabine and cedazuridine (Inqovi)• Approved in 2020 for previously treated and untreated, de novo and
secondary MDS with FAB sub-types: RA, RARS, RAEB and CMML and intermediate-2 and high-risk IPSS
• Administered on Days 1-5 of a 28-day cycle
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Garcia-Manero G, et al. Blood 2020
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MDS TREATMENT Other Treatments
• Venetoclax• BCL-2 inhibitor• Approved for CLL and relapsed AML• Can be used off label in conjunction with hypomethylating agents in patients with
high-risk disease • Given x 14 days out of 28-day cycles
• IDH inhibitors • Ivosidenib (Tibsovo) – IDH1 inhibitor
• Approved for newly diagnosed AML (>75 years or age) unfit for intensive chemotherapy with IDH1 mutation
• Approved for R/R AML with IDH1 mutation• Enasidenib (Idhifa) – ID2 inhibitor
• Approved for R/R AML with IDH2 mutation
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Hematopoietic Stem Cell Transplant (HSCT)• High risk subtypes of MDS with good performance status• Only curative therapy available to patients with MDS• P53 data often do poorly• Important to risk-stratify with transplant MD• Meet transplant doctor up-front if a high risk patient • Important to consider performance status, especially as many of our
patients are older adults• Social work consult to help determine if appropriate support is in place• Discuss with patient what their individual goals• Clearly outline risk of transplant
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Lindsley et al. N Engl J Med 2017; 376:536-547
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Clinical Trials in MDS: Why ? • Trials can be for upfront new diagnosis, post transplant, and relapsed / refractory MDS. • Important in the development of new treatments • Patients may have opportunity to get treatment that isn’t available to everyone and
may benefit from it. • Participating in clinical trials requires more visits and evaluation.
• Many trials allow SOC treatments to be given locally like HMA and lab checks• Patients are usually required to be at trial site on required study drug visits such
administration of agent, PK/ PD testing and disease assessments.• Some trials reimburse for travel, hotels or mileage.• Sponsors only cover the study drug and any extra assessments associated with it
that are not considered SOC. • clinicaltrials.gov - database of clinical trials that are required to be registered,
maintained by the NIH
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Clinical Trials in MDS Examples of Clinical trials include:
o CPX-351 ( vyxeos ) for Higher risk MDS post HMA ( phase 1/2)o AG-120 for IDH mutated MDS (phase 2) o AZD5991 for r/r MDS ( phase 1)o JNJ-67571244 in r/r MDS ( phase 1) o TP-0184 for anemia ( phase 1)o LY3410738 for IDH 1 or IDH 2 MDS (phase 1) o Sea-CD70 in r/r MDS ( phase 1) o Magrolimab/ Placebo +AZA for MDS (phase 3)o MBG453/ placebo +AZA for HR MDS (phase3) o APR-246 + Azacitidine for the Treatment of TP53 Mutant MDS (Phase 3)
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MBG-453• MBG 453 ( sabatolimab) is an anti TIM 3 checkpoint inhibitor
• Given intravenously• Phase 1b trial of MBG453 ( day 8 and 22) with HMA ( azacitidine days 1-
7 or decitabine 1-5 results showed: • 62.9 % overall response rate• Time to response was 2 months
• Side Effects seen in combination with HMA : thrombocytopenia, neutropenia and anemia • Including immune related AE’s including increasing LFT’s, arthritis,
hepatitis, hypothyroidism and rash. • Currently there is an ongoing phase 3 trial of
MBG 453 / placebo with Azacitidine
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Magrolimab• Magrolimab is an Anti-CD47 monoclonal antibody
• Given as intravenously • The FDA granted Breakthrough Therapy based on positive results of Phase 1b
study, which evaluated magrolimab w/ azacitidine in previously untreated intermediate, high and very high-risk MDS.• 42% of MDS patients achieved a CR.• Median response rate was noted to be at 1.9 months• RBC transfusion independence seen in approx 56%• subgroup analysis is looking into those with Tp53 mtutation
• Side effects include anemia fatigue, infusion reaction, neutropenia and thrombocytopenia• anemia was mitigated by first giving patients a priming (low) dose of
magrolimab before giving them maintenance dose
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APR-246 (eprenetapopt) ● APR-246 ( eprenetapopt) - reactivates mutant p53-thereby triggering
programmed cell death in human cancer cells.● Given intravenously over 6hrs
● FDA granted it breakthrough therapy and fast track designations based on results of phase 1/2 studies of APR246 in combination w/ Azacitadine for treatment of upfront Tp53 mutated MDS ● APR-246 press release in 12/2020 stated that data didn’t show CR rate better
than AZA alone but data analysis is ongoing ● Apr 246 arm showed response rate higher but not statistically significant.
● Some ide effects seen in combination: nausea/ vomiting , fatigue, thrombocytopenia, neutropenia and including neurological side effects such as ataxia, confusion, dizziness and facial paresthesia
● APR-548 - is an oral p53 reactivator also being developed by Aprea
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CASE STUDY: MR. F
• 78 y.o Male• Diagnosis of MDS –RS• R-IPSS Score: 2 (low risk) = 5.3 years • H/H: 8.2/ 24.8• Epo level > 1400• Requiring blood transfusions with 2 units PRBCs every 6 weeks
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• What are some appropriate therapy options that may be offered to Mr. F? Select all that apply:
A) ESA therapy (epoetin or darbepoetin)B) HMA therapy (decitabine or azacitidine)C) HMA therapy as a bridge to stem cell transplantD) Transfusions alone
POLLING QUESTION:
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CASE STUDY: MR. F
• December 2017: Decided to continue transfusion support• Plans for clinic follow up every 6 weeks for labs and red blood cell
transfusions • By April 2018: ,his red cell transfusion needs have overall increased, and he
is feeling less benefit in symptoms after each transfusion• Started chemotherapy treatment with 3-day decitabine in hopes this will
improve his cytopenia's and decrease transfusion needs• In May 2018 during his first cycle of decitabine he was admitted for fever
and neutropenia and was found to have pneumonia. • Was in the ICU required dialysis• After fully recovering he opts for transfusion support alone.
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CASE STUDY: MRS. L
• WBC: 1.71• HCT: 22.1• PLT: 36• ANC: 370• Blasts: 9%
• AGE : 82 y.o• Cytogenetics: complex karyotype• Molecular: TP53 mutation• R-IPSS: 8.5- Very high risk
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POLLING QUESTION:
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CASE STUDY: MRS. L
• January 2021: started a clinical trial with HMA combination therapy• Azacitidine 75mg/m2 on Days 1-7 • MBG453 400mg IVB on Days 8 and Day 22
• She continued to require transfusions and during cycle 1 needed them twice weekly
• During cycle 2• disappearance of peripheral blasts• transfusion needs less (PLTs >25 and HCT >26)
• Able to travel to Maine with her husband for a long weekend and continues to have some fatigue but overall feels better
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Factors that Help Determine Treatment Options and Impact Approach to Care
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Quality of Life Issues Surrounding Care of MDS Patients and Families
Diagnosis
● Helping patient and care providers through the initial shock of diagnosis + fear of what the diagnosis means
● Setting realistic expectations that often treatment is non-curative● Aiding patient and care providers with adjusting to new normal
Assess
Consult Additional Services
● Social work● Nutrition● Oncology Psychologist
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Nursing Implications ● Patients need to be educated about symptoms prior to start of treatment and
ongoing during care● Educate on the management of fatigue, nausea, pancytopenia
■ anemia - can cause fatigue, SOB, dizziness ■ bleeding risk- increases when platelets are low. Including nose bleeds
and bruising and when to call ■ neutropenia- risk for infection including sign and symptoms, possibility
of have fevers and what to do or who should they call ■ nausea - use of antiemetics, small frequent meals ■ fatigue - discuss ways to manage fatigue with naps■ risk for falls- this may be related to anemia, dizziness, dehydration etc
● Educating regarding length of treatment -this means frequent visits, transfusion needs, and importance of coming in for their evaluations
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Nursing implications continued● Line care - many will end up needing a central line due to need for transfusion
and treatment.
● MDS patients tend to be older - evaluating performance and cognitive status ■ these patient also may have multiple comorbidities. ■ who is the patient's primary family support
● Evaluate psychosocial status including - financial, spiritual , evenviroment, coping resources, where they live etc.
● Patients will likely require multiple bone marrow biopsy - patients will have anxiety, stress and pain
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Serious Illness Conversations ● Importance of initial assessment
● Ongoing conversation
● Across care continuum
● Responsibility of each care team members
● Address quality of life issues and goal of care in real time
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- After Mr. F was discharged from ICU he did transfusions only
- July 2019 - After much discussion he started lenalidomide and continued it for 4 cycles.
- By Feb 2020- he had completed 4 cycles of lenalidomide but his bm bx revealed with progressive disease to MDS EB2.- He continued with transfusion support at that time
- May 2020 he was started on luspatercept. - Bm bx showed persistent disease with 10% blasts in September 2020
- In September 2020 he was enrolled to a clinical trial using magrolimab. - Trial initially started with magrolimab only and then HMA could be
added if needed at cycle 3
Case Study: Mr F
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- He had abscess infection and was admitted during cycle 2 for abx. - Bm bx was performed at the end of the 2nd cycle showed stable
disease. - Mr. F started cycle 3. Trial allowed vidaza to be added to his
Magrolimab. - During that cycle he continued to become weaker and decline at
home. - After much discussion he came off trial and was bridge to hospice in
December 2020.
- He passed away at home surrounded by his family in early January.
Case Study: Mr. F
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POLLING QUESTION:
In caring for the patient with MDS. What do you think is the most challenging aspect of their care?
A) Educating regarding the diagnosis B) The management and education of symptoms they may have C) The psychosocial challenges that chronic dx bringsD) The limited lack of options for treatment
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POLLING QUESTION:
In thinking about the topic of serious illness conversations, what are appropriate questions to assess patients awareness or prognosis?
A. What is your understanding of your illness?B. What are your hopes about your health?C. What are your worries?D. All of the above
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Community Care for Patients with MDS● Collaboration● Communication● Close and Ongoing follow up● Incorporation of primary care providers and other members of
care team ● Improving and focusing on quality of life● Telemedicine and its impact
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What is Still Needed?
● More treatment
● Clinical trials
● Tools for tracking transfusion needs
● More resources for patients and providers, such asthe MDS Toolkit from AAMDSIF
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AAMDSIF MDS Toolkit
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Thank you