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Myelodysplastic Syndromes (MDS):Diagnosis, Treatment, and Side Effects Management
Learning Objectives
• Describe the various types and subtypes of MDS
• Identify tests used to diagnose disease and monitor treatment of MDS
• Explain the overarching goals of treatment for the types of MDS
• Explain approved and emerging treatment options for MDS, including stem cell transplantation, and the role of clinical trials
• Describe the roles of the pharmacist, the nurse and the social worker in treating patients with MDS
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Faculty
Sangmin Lee, MDAssistant Professor of Medicine
Weill Cornell Medical College
Assistant Attending Physician
New York-Presbyterian Hospital
New York, NY
Peter Campbell, PharmD, BCOPClinical Pharmacy Manager,
Hematology/OncologyColumbia University Irving Medical Center
New York, NY
Ayelet Nelson, MSW, ANP-BCAdult Care Nurse Practitioner
Weill Cornell MedicineNew York, NY
Myelodysplastic Syndrome (MDS)
Sangmin Lee, MD
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What Is MDS?
• MDS = myelodysplastic syndrome
• Group of heterogeneous clonal bone marrow disorders with dysplastic and ineffective hematopoiesis and increased risk of transformation to leukemia
• Most commonly diagnosed myeloid neoplasm in U.S.
– Incidence: 4.6/100,000
• Predominantly disease of the elderly
– ~86% patients are older than 60 at diagnosis
Diagnostic Workup for MDS
• Complete blood count with (manual differential), serum erythropoietin
level in certain cases
• Bone marrow biopsy/aspirate
o Morphology
─ Dysplasia, blasts, ring sideroblasts
o Flow cytometry
o Cytogenetics
─ Evaluate for cytogenetic abnormalities associated with MDS
o Mutation testing
─ Most often myeloid mutation panel using next-generation
sequencing
─ Prognostic, potentially therapeutic implications
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MDS is a Heterogeneous Disease
• 2016 WHO classification:
– MDS with single lineage dysplasia (MDS-SLD)
– MDS with ring sideroblasts (MDS-RS)
– MDS with multilineage dysplasia (MDS-MLD)
– MDS with excess blasts-1(MDS-EB-1)
– MDS with excess blasts-2 (MDS-EB-2)
– MDS, unclassifiable (MDS-U)
– MDS with isolated del(5q)
– Refractory anemia of childhood
Arber, et al. Blood 2016
Predisposing Factors for MDS
• Prior chemotherapy
• Environmental (chemicals, benzene, radiation, tobacco)
• Inherited genetic abnormalities
• Familial MDS
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Clinical Course of MDS is
Heterogeneous
IPSS-R: cytogenetics, marrow blasts, cytopenias
Survival based on risk (IPSS-R) AML transformation based on risk
Greenberg et al. Blood. 2012.
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IPSS-R
Greenberg, et al. Blood 2012
Prognostic variableScore
0 0.5 1 1.5 2 3 4
Cytogenetics Very good Good Intermediate Poor Very poor
Bone marrow blast
(percent)≤2 >2 to <5 5 to 10 >10
Hemoglobin (g/dL) ≥10 8 to <10 <8
Platelets (cells/microL) ≥100 50 to <100 <50
Absolute neutrophil count
(cells/microL)≥0.8 <0.8
Risk group IPSS-R score
Very low ≤1.5
Low >1.5 to 3.0
Intermediate >3 to 4.5
High >4.5 to 6
Very high >6
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General Treatment Goals in MDS
• “Lower risk MDS”
– Improve transfusion dependence
• “Higher risk MDS”
– Improve overall survival, prevent progression to AML
Current Treatment Approaches
for MDS
• “Lower risk MDS”o Supportive care with transfusionso Growth factorso Lenalidomide (Revlimid®) for del(5q)o Hypomethylating agentso Immunosuppressive therapies for hypocellular MDS
• “Higher risk MDS”o Hypomethylating agentso Chemotherapyo Allogeneic transplant for younger patients
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Erythropoiesis Stimulating
Agents in MDS
• Anemia present in >80% of MDS patients at diagnosis
• Transfusions associated with significant morbidity
• Epoetin alfa (Epogen®, Procrit®) or darbepoetin alfa (Aranesp®)
• Various dosing/schedules for either agent
• Hematologic response 20-40%, usually in 12-16 weeks
• Higher response rate with ESA + G-CSF if erythropoietin ≤500 mU/mL and transfusions <2 U/month
• Side effects: hypertension, fever, headache, nausea, chest pain
Hellstrom-Lindberg E, Blood 1998, Musto P Br J Haematolo 2005
Lenalidomide (Revlimid®) in
Lower Risk MDS
• MDS with del(5q): red cell transfusion independence in ~67%
• Given 10 mg/day for 21 days every month or continuous daily
• Median time to response ~4.6 weeks
• Median duration of transfusion independence >104 weeks
• MDS without del(5q): red cell transfusion independence in ~26%
• Most common side effects: neutropenia and thrombocytopenia
List. NEJM. 2006.
Raza. Blood. 2008.
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On the Horizon: Luspatercept (Reblozyl®) in
Lower Risk MDS: MEDALIST Trial
• Increased TGF ligands linked to ineffective erythropoiesis in MDS
• Luspatercept (Reblozyl®): TGF inhibitor
• MEDALIST Trial: Phase III randomized double blinded placebo study of
luspatercept (Reblozyl®) in lower risk MDS (HMA naive)
o MDS-RS (WHO): ≥15% ringed sideroblasts or ≥5% with
SF3B1 mutation
• Subcutaneously 1.0 mg/kg every 21 days (titrated to max 1.75 mg/kg)
• Primary endpoint:
o Red cell transfusion independence ≥8 weeks during first 24 weeks
─ 37.9% vs 13.2% (p<0.0001)
• Most common side effects: fatigue (27%), diarrhea (22%),
asthenia (20%), nausea (20%)
List. ASH. 2018.
MEDALIST Trial: Durable RBC
Transfusion Independence
Reblozyl® is the brand name for luspatercept.
List. ASH. 2018.
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Hypomethylating Agents in MDS
Baylin SB. Nat Clin Pract Oncol. 2005.
AZA-001: Azacitidine (Vidaza®) vs
Conventional Care in High Risk MDS
Fenaux et al. Lancet Oncol. 2009.
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Azacitidine (Vidaza®) Up-Front Prolongs
Overall Survival in MDS
AZA-001
OS: 21.1 vs 11.5 mo (p=0.0045)
Fenaux et al. Lancet Oncol. 2009.
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AZA-001 continued
• Azacitidine (Vidaza®) given 75mg/m2 subcutaneous (or intravenously) for 7 days every 28 days
• Azacitidine (Vidaza®) given median 9 cycles
• Azacitidine (Vidaza®)vs conventional care:– Complete remission: 17% vs 8% (p=0.015)
– Hematologic improvement: 49% vs 29% (p<0.0001)
– Erythroid improvement: 40% vs 11% (p<0.0001)
– Platelet improvement: 33% vs 14% (p=0.0003)
– Neutrophil improvement: 19% vs 18% (p=0.87)
– Decreased infections requiring IV antibiotics by 33%
• Most common side effects: cytopenias, injection site reaction, nausea, vomiting, fatigue, diarrhea
Fenaux, et al. Lancet Oncology 2009;10: 223-32
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Decitabine (Dacogen®) in
High-risk MDS
• Phase III randomized study of decitabine vs
supportive care:
– CR: 9% vs 0%, p<0.001
– Hematologic improvement: 13% vs 7% p<0.001
– Overall improvement (CR+PR+HI): 30% vs 7% p<0.001
– Time to AML or death: 12.1 mo vs 7.8 mo (p=0.16)
Kantarjian Cancer 2006
Azacitidine (Vidaza®) Prolongs Survival in
High-Risk MDS but Outcome Is Dismal
After Azacitidine (Vidaza®) Failure
Median overall survival 4-6 months
Prebet et al. JCO. 2011.
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Continued Azacitidine (Vidaza®) in
Stable Disease May Be Beneficial
Papageorgiou SG. Hematol Oncol. 2018.
How About Decitabine (Dacogen®)
After Azacitidine (Vidaza®)?
• Response low (ORR ~19% in one study, no CR)
• Responses short (2-5 months)
• Survival short (5.9-7.3 months)
Harel et al. Leuk Res. 2015.
Duong et al. Leuk Lymphoma. 2015.
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HMA in Lower Risk MDS
• Azacitidine (Vidaza®):
– Azacitidine (Vidaza®) 75mg/m2 SC for 5 days
– 38% Hematologic improvement, 19% CR, 65% stable
disease
• Decitabine (Dacogen®):
– Decitabine (Dacogen®) 20mg/m2 SC for 3 days
– Overall improvement rate (CR, PR, HI): 23%,
Hematologic improvement: 7%, 67% RBC/platelet
transfusion independent
Fili et al, Clin Cancer Research 2013, and Garcia-Manero, et al JCO 2013
“Lower Risk” MDS Has Poor Outlook
Post-HMA
Jabbour et al. Cancer. 2015.
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No Standard Approach to Post-HMA
Therapy in MDS• For healthy, fit patients:
– Intensive chemotherapy, SCT
• Majority of MDS patients are not candidates for intensive chemotherapy or stem cell transplant
• Large phase III trial: rigosertib (Estybon®) vs best supportive care– OS: 8.2 months vs 5.8 months (p=0.27)
• Effective treatment after HMA therapy is an unmet need in MDS
• Consider clinical trials when possible!Manero et al, Lancet Oncol. 2016 Apr;17(4):496-508.
Myelodysplastic Syndrome (MDS)The Pharmacist’s Role in Treatment and
Side Effects Management
Peter Campbell, PharmD, BCOPClinical Pharmacy Manager, Hematology/Oncology
Columbia University Irving Medical CenterNew York, NY
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Pharmacist
Role
Prior
Authorizations
Chemotherapy
Counseling
Dose
Modifications
Discharge
Preparation
Medication Review Supportive Care
Chemotherapy
Selection
Dose
Modifications
Toxicity Checks
Patient
Counseling
Antibiotic
Recommendations
Therapeutic Drug
Monitoring
Patient Case
• JM is a 78-year-old male with a past medical history of hypertension (HTN), hyperlipidemia (HLD), non-insulin dependent diabetes, COPD, and single knee replacement in 2016 who presents to the clinic after a month of increasing fatigue and easy bruising. JM’s labs resulted with a WBC of 4.1, ANC of 800, hemoglobin of 8.1, and platelet count of 210,000. A bone marrow biopsy was performed which was consistent with MDS with 3% immature myeloid cells, no mutations identified, del(5q) present
• What would be the preferred therapy for this patient?
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Treatment Options
• Supportive care– Transfusion support
– Iron chelation
– Erythropoiesis-stimulating agents (epoetin alfa [Epogen®, Procrit®], darbepoetin alfa [Aranesp®])
• Low intensity therapy– Hypomethylating agents (azacytidine [Vidaza®],
decitabine [Dacogen®])
– Biological response modifiers (lenalidomide [Revlimid®])
– Immunosuppressive therapy (anti-thymocyte globulin [ATGAM®]) cyclosporine (Sandimmune®, Gengraf®, Neoral®, and Restasis®)
• High intensity therapy– Remission-induction therapy [idarubicin (Idamycin®),
cytarabine (Cytosar-U®), or fludarabine (Fludara®) based]
NCCN. Myelodysplastic Syndromes Guidelines. Version 2.2019.
Iron Chelation Therapy
Deferoxamine (Desferal®) Deferasirox (Jadenu®) Deferasirox (Exjade®)
Administration
• SubQ: 5–7 days per week • Granules: sprinkle on soft food immediately prior to administration, on an empty stomach or with light meal
• Tablets: take with water or other liquids, on an empty stomach or with light meal
• Tablets for suspension: make an oral suspension using water, apple juice, or orange juice, stirred to a fine foam. Take at least 30 minutes prior to food.
*Please refer to product-specific prescribing information for full administration instructions
Adverse effects
• Infusion site reactions• Hearing abnormalities• Visual disturbances
• Upset stomach• Nephrotoxicity• Hepatotoxicity• GI hemorrhage
Other information
• Topical anesthetics or corticosteroids can be used for injection site reactions
• Contraindicated in high-risk MDS
Leuk Res. 2015;39(10):1028-33.
Jadenu (deferasirox) [prescribing information]. East Hanover, NJ: Novartis Pharmaceuticals; December 2018.
Exjade (deferasirox) [product monograph]. Dorval, Quebec, Canada: Novartis Pharmaceuticals Canada Inc; November 2018.
Desferal (deferoxamine) [prescribing information]. East Hanover, NJ: Novartis Pharmaceuticals Corporation; April 2018.
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Erythropoiesis-Stimulating Agents
• Although these agents are used in this setting, they do not alter the natural course of the disease– Studies have shown that there is not an increased risk of
progression to acute myeloid leukemia
• Patients that will have the best response are those with low baseline erythropoietin (EPO) levels– Typically response is greatest when EPO <500 units/L
• A hemoglobin of 10–12 g/dL should be targeted to reduce transfusions– Do NOT try to target a hemoglobin >12 g/dL
• In 2017 the FDA removed the Risk Evaluation and Mitigation Strategy (REMS) program for both darbepoetin alfa (Aranesp®) and epoetin alfa (Epogen®, Procrit®)
• In select scenarios, the use of G-CSF may improve the efficacy of erythropoiesis-stimulating agents
NCCN. Myelodysplastic Syndromes Guidelines. Version 2.2019.
Erythropoiesis-stimulating agents
Epoetin Alfa
(Epogen®, Procrit®)
• Dosing
– 150 – 300 units/kg SubQ
TIW
• Pharmacokinetics
– Onset: 10 days
– Peak: 2 – 6 weeks
– Half-life: 16 – 67 hours
Darbepoetin Alfa
(Aranesp®)
• Dosing
– 150 – 300 mcg SubQ once
weekly OR 500 mcg every
2 – 3 weeks
• Pharmacokinetics
– Onset: 2 – 6 weeks
– Half-life: approximately 3
times longer than epoetin
Data suggests that darbepoetin and epoetin have similar efficacy, with darbepoetin possibly resulting in improved outcomes due to the prolonged half-life
Epogen (epoetin alfa) [prescribing information]. Thousand Oaks, CA: Amgen; July 2018.
Aranesp (darbepoetin alfa) [prescribing information]. Thousand Oaks, CA: Amgen Inc; January 2018.
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Hypomethylating Agents
• Agents in this class increase hypomethylation of DNA, which allows normal gene differentiation and proliferation to be restored
• Azacitidine (Vidaza®) and decitabine (Dacogen®) are generally thought of as equally efficacious for treatment outcomes– Azacitidine has shown improved survival in high-risk patients with
MDS, and may be preferred in this setting
• Patients should receive 4 – 6 courses of therapy prior to determining treatment failure– Patients that are responding to therapy should continue to receive
treatment until no longer responsive
– Dosing frequency can be extended if treatment-related toxicities occur
NCCN. Myelodysplastic Syndromes Guidelines. Version 2.2019.
Azacitidine (Vidaza®)
• Dosing– 75 mg/m2/day for 7 days every 4 weeks
• May be given Mon – Fri, rest Sat/Sun, and completed Mon – Tues
• Administration– Must be infused within 1 hour of reconstitution due to stability
– Infuse over 10–40 minutes
• Dose adjustments– Neutropenia, thrombocytopenia (treatment-related effects)
– Renal impairment (renal clearance of the drug and metabolites)
• Adverse effects– Myelosuppression, hepatotoxicity, injection site reactions,
nausea/vomiting (moderate emetic potential)
Vidaza (azacytidine) [prescribing information]. Summit, NJ: Celgene Corporation; September 2018.
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Decitabine (Dacogen®)
• Various dosing schedules
– 15 mg/m2 every 8 hours for 3 days every 6 weeks, OR
– 20 mg/m2 daily for 5 days every 28 days
• Administration
– Infuse over 1–3 hours, depending on regimen design
• Dose adjustments
– Myelosuppression, serum creatinine elevations, liver function
test abnormalities, uncontrolled infections
• Adverse events
– Myelosuppression, fatigue, dizziness, constipation,
nausea/vomiting (minimal emetic potential)
Dacogen (decitabine) [prescribing information]. Rockville, MD: Otsuka America Pharmaceutical; April 2016.
Lenalidomide (Revlimid®)
• Selectively inhibits secretion of pro-inflammatory cytokines along with other immunomodulatory, antiangiogenic, and antineoplastic mechanisms
• Dosing– 10 mg by mouth once daily, with water, at approximately the same time each day
• Dose adjustments– Renal impairment (renal clearance of the drug)
– Thrombocytopenia, neutropenia (treatment-related effects)
• Adverse effects– Myelosuppression, drowsiness, neuropathies
– Black Box Warning: arterial and venous thrombotic events
• REMS program (male and female patients)– Requires routine pregnancy testing for females of reproductive potential prior to and
during treatment
– Females must always use 1 highly effective and 1 additional effective birth control method, while males must always use a latex/synthetic condoms
• Precautions must be taken for at least 4 weeks following stopping lenalidomide (Revlimid®)
– Refer to full REMs information for additional detailed process and documentation requirements
Revlimid (lenalidomide) [prescribing information]. Mississauga, Ontario, Canada; Celgene Inc; November 2018.
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Anti-Thymocyte Globulin, Equine
(ATGAM®)
• Mechanism– Alters or inactivates antigen-reactive T lymphocytes
• Administer over at least 4 hours– Not typically titrated, as is done in other indications
– Preferred through a central line
• Equine anti-thymocyte globulin (ATGAM®) may be preferred over rabbit anti-thymocyte globulin (Thymoglobulin®)
• Serum sickness– Immune complex mediated hypersensitivity reaction
– Typically has a delayed onset of days to weeks
– Symptoms include fevers, rash, and arthralgias
– Treatment includes drug withdrawal or corticosteroids• Prednisone 0.5-1 mg/kg once daily, with rapid taper
– If recurrence, steroid may be re-initiated
Leukemia. 2004;18:460-65.
J Clin Oncol. 2011;29:303-309.
Haematologica. 2014;99(9):1433-40.
Anti-Thymocyte Globulin, Equine
(ATGAM®)
• Premedications– Antihistamine, corticosteroid, and an antipyretic
– Consider withholding beta-blockers
• Test dose– Initial epicutaneous prick of undiluted ATG (ATGAM®)
– If no wheal in 10 minutes, give 0.02 mL of 1:1000 dilution of ATG (ATGAM®) along with a 0.02 mL control and observe for 10 minutes more
• Wheal with initial skin prick or ≥3 mm larger than control indicates a positive reaction
– Positive skin tests indicate high likelihood of an infusion reaction
– A negative skin test does NOT mean that a patient will not have anaphylaxis
– Medications should be available to treat anaphylactic reactions
ATGAM (lymphocyte immune globulin, antithymocyte globulin [equine]) [prescribing information]. New York, NY: Pfizer Inc; May 2018.
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Cyclosporine (Sandimmune®, Gengraf®,
Neoral®, and Restasis®)
• Mechanism
– Binds to intracellular cyclophilin and inhibits the calcineurin
complex to inhibit interleukin II to inhibit T lymphocytes
• Dosing
– Approximately 5 mg/kg/day in divided doses
• Trough levels
– Target approximately 200 ng/mL
• Adverse effects
– Afferent arteriole vasospasm, magnesium wasting, QTc
prolongation, tremors, headaches, hypertension, hyperlipidemia,
infection, secondary malignancies, PTLD1
Gengraf (cyclosporine) [prescribing information]. North Chicago, IL: AbbVie Inc; February 2017.
Neoral (cyclosporine) [prescribing information]. East Hanover, NJ: Novartis; March 2015.
Sandimmune (cyclosporine) [prescribing information] . East Hanover, NJ: Novartis; March 2015.1 Posttransplant lymphoproliferative disease
Cyclosporine (Sandimmune®, Gengraf®,
Neoral®, and Restasis®)
Sandimmune®(non-modified)
Neoral®(modified)
Gengraf® (Modified)
• Original formulation• Bile-dependent
absorption (poor, variable)
• High interpatient variability in drug exposure
• Microemulsion• Bile-independent
absorption• Increased systemic
absorption, bioavailability
• AB-rated, bioequivalent formulation of Neoral®
• Caution: 80-125% bioequivalence acceptance range
Gengraf (cyclosporine) [prescribing information]. North Chicago, IL: AbbVie Inc; February 2017.
Neoral (cyclosporine) [prescribing information]. East Hanover, NJ: Novartis; March 2015.
Sandimmune (cyclosporine) [prescribing information] . East Hanover, NJ: Novartis; March 2015.
Not interchangeable
Interchangeable
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High-Intensity Therapy
• Used in patients with MDS that is prognostically similar
to acute myeloid leukemia
• Recommended only in the context of a clinical trial
• Idarubicin (Idamycin®)-based regimens
– Idarubicin (Idamycin®) plus cytarabine (Cytosar-U®)
– Regimens with idarubicin (Idamycin®) have shown to have
improved outcomes when compared to regimens with other
agents
• Fludarabine-based regimens
– Fludarabine (Fludara®) plus cytarabine (Cytosar-U®)
Blood. 2001;98;3575-83.
NCCN. Myelodysplastic Syndromes Guidelines. Version 2.2019.
Patient Case
• JM is a 78-year-old male with a past medical history of HTN,
HLD, non-insulin dependent diabetes, COPD, and single
knee replacement in 2016 who presents to the clinic after a
month of increasing fatigue and easy bruising. JM’s labs
resulted with a WBC of 4.1, ANC of 800, hemoglobin of 8.1,
and platelet count of 210,000. A bone marrow biopsy was
performed which was consistent with MDS with 3% immature
myeloid cells, no mutations identified, del(5q) present
• What would be the preferred therapy for this patient?
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Patient Case
• JM is a 78-year-old male with a past medical history of
HTN, HLD, non-insulin dependent diabetes, COPD, and
single knee replacement in 2016 who presents to the
clinic after a month of increasing fatigue and easy
bruising. JM’s labs resulted with a WBC of 4.1, ANC
of 800, hemoglobin of 8.1, and platelet count of 210,000.
A bone marrow biopsy was performed which was
consistent with MDS with 3% immature myeloid cells, no
mutations identified, del(5q) present
Patient Case
• JM is a 78-year-old male with a past medical history of
HTN, HLD, non-insulin dependent diabetes, COPD, and
single knee replacement in 2016 who presents to the
clinic after a month of increasing fatigue and easy
bruising. JM’s labs resulted with a WBC of 4.1, ANC
of 800, hemoglobin of 8.1, and platelet count of 210,000.
A bone marrow biopsy was performed which was
consistent with MDS with 3% immature myeloid cells, no
mutations identified, del(5q) present
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Patient Case
• JM is a 78-year-old male with a past medical history of
HTN, HLD, non-insulin dependent diabetes, COPD, and
single knee replacement in 2016 who presents to the
clinic after a month of increasing fatigue and easy
bruising. JM’s labs resulted with a WBC of 4.1, ANC
of 800, hemoglobin of 8.1, and platelet count of 210,000.
A bone marrow biopsy was performed which was
consistent with MDS with 3% immature myeloid cells, no
mutations identified, del(5q) present
Patient Case
• JM is a 78-year-old male with a past medical history of
HTN, HLD, non-insulin dependent diabetes, COPD, and
single knee replacement in 2016 who presents to the
clinic after a month of increasing fatigue and easy
bruising. JM’s labs resulted with a WBC of 4.1, ANC
of 800, hemoglobin of 8.1, and platelet count of 210,000.
A bone marrow biopsy was performed which was
consistent with MDS with 3% immature myeloid cells, no
mutations identified, del(5q) present
• Lenalidomide (Revlimid®) 10 mg once daily
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The Roles of the Nurse, Nurse Practitioner, and Social Worker in the Treatment of Patients With MDS
Ayelet Nelson, MSW, ANP-BCAdult Care Nurse Practitioner
Weill Cornell MedicineNew York, NY
Biopsychosocial Approach to Care
Psychological:
Coping Skills
Mental Health
Self-Esteem
Family Relationships
Adherence
“New Normal”
Biological:Age
DiseaseSide effects
ComorbiditiesTreatment
Treatment Goals
Social:Quality of Life
Caregiver supportFriends and family
supportFinancial Support
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Biopsychosocial Approach To Care
• Biological:
– Explanation of disease in terms a patient can understand and digest (at diagnosis and throughout treatment course)
• i.e. What is the bone marrow? What does dysplastic mean? What is the function of white cells?
– Elaborating on treatment rationale
– Education around treatment and side effects
Biopsychosocial Approach To Care (Continued)
• Biological:
– Management of side effects from disease and treatment
– Identifying new or changing symptoms
– Ongoing explanation of blood tests, radiology studies, pathology/bone marrow reports
– Perform bone marrow biopsies (NP)
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Blood cancers can develop in many different places within normal blood cell formation.The type of blood cancer that results has to do with where normal cell development is blocked.
This picture shows the cell type where different blood cancers arise.
• Chronic lymphocytic leukemia (CLL)
• B-cell non-Hodgkin lymphoma
• Hairy cell leukemia
• Hodgkin lymphoma
• T-cell non-Hodgkin lymphoma
• T-cell large granular lymphocytic (LGL) leukemia
• NK-cell non-Hodgkin lymphoma
• NK-cell large granular lymphocytic (LGL) leukemia
Lymphoid stem cells
Blast cells
Lymphocytes
B lymphocytes T lymphocytes Natural killer cells
• Acute lymphoblastic leukemia (ALL)
Myeloid stem cells
Various precursor or blast cells
Mature cells
• Chronic myeloid leukemia (CML)• Myeloproliferative neoplasms (MPNs)
Myelofibrosis (MF)Polycythemia vera (PV)Essential thrombocythemia (ET)
• Chronic myelomonocytic leukemia (CMML) and juvenile myelomonocytic leukemia (JMML)
Plasma cells
• Myeloma
Bone forming stem cells
• Myelodysplastic syndromes• Acute myeloid leukemia (AML)
From The Leukemia & Lymphoma Society website. https://www.lls.org/resource-center/download-or-order-free-publications
Common Side Effects of MDSand Treatment of MDS
• Cytopenias (neutropenia, anemia, thrombocytopenia)
• Fatigue
• GI toxicities
• Poor appetite
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Neutropenia• Provide education related to signs and symptoms
of infection
• When to call the office; when to go immediately to the emergency room
• Prophylactic antimicrobials (NP)
• Neutropenic precautions (eg, good hand-washing, avoiding sick contacts)
• Growth factors (NP)
Anemia
• Provide education regarding signs and symptoms of anemia
• Fatigue is the most commonly reported symptom in patients with anemia
• Red cell growth factors (NP)
• When to transfuse
• Transfusion complications: infusion reaction, development of antibodies
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Thrombocytopenia
• Provide education regarding signs and symptoms of bleeding, lifestyle changes
• Identification of when transfusion is indicated
• Transfusion complications: infusion reaction, development of antibodies
Fatigue• One of the most difficult side effects to treat
– Blood is not always the answer
– Encouraging light exercise (eg, walking)
– Listening to your body
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Gastrointestinal Toxicity
• Nausea: Identify and treat EARLY!
– Side effect of specific treatments
– Choosing an antiemetic (NP)
• Constipation: Identify and treat EARLY!
– Side effect of specific treatments
– Prevention vs treatment
• Diarrhea: Identify and treat EARLY!
– Side effect of specific treatments
– Electrolyte imbalances
– Test for infectious process before treating
Poor Appetite
– Identify if it is poor appetite or nausea
– High calorie foods
– High protein foods
– Maximizing every bite
– Grazing
– Nutrition consult
– Medical marijuana
– Mirtazapine/Quetiapine
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Treatment Goals
• Clarification of goals of therapy – Depends on stage of disease and age/comorbidities– Transfusion independence – Prevent transformation to AML
• Ongoing discussion of disease status and treatment updates
• Minimize/manage toxicities • Improve/maintain quality of life • Collaboration with clinical trial staff• “New normal”
Biopsychosocial Approach to Care
• Psychological:
– Act as a confidant for patients and liaison to multidisciplinary team
– Emotional support for patients and their caregivers
– Identifying stressors and assisting with management
– Mental health referrals: the earlier the better
– Addressing and collaboration to increase adherence to medications (including oral chemotherapy drugs as well as supportive medications)
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Biopsychosocial Approach To Care
• Social:
– Loss of job, autonomy, daily routine
– Change of role in family unit and in other arenas
– Appropriate referrals to organizations for support
– Referral to social work
Overview of Nursing Role• Take into consideration the “whole” person
• Partner with patient and caregivers to promote optimal care with mutual understanding of goals
• Communicate in ways that meet the patient and caregivers where they are
• Use terms that everyone in the room understands
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The Social Worker’s Role• Establish therapeutic relationship with social worker early on
– Counseling for patient and support network throughout care
– Exploring impact of the “new normal”
» Sword of Damocles
– Access to durable medical equipment to assist with ADLs
– Referral to appropriate support groups, organizations
– Financial assistance for copays, medication, loss of income
Revisiting Case Study: JM
JM is a 78-year-old male with a past medical history of hypertension (HTN), hyperlipidemia (HLD), noninsulin-dependent diabetes, COPD, and single knee replacement in 2016 who presents to the clinic after a month of increasing fatigue and easy bruising. JM’s labs resulted with a WBC of 4.1, ANC of 800, hemoglobin of 8.1, and platelet count of 210,000. A bone marrow biopsy was performed that was consistent with MDS with 3% immature myeloid cells, no mutations identified, Del5q present
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What is Significant in This Case When Thinking About Next Steps From a
Nursing and Social Work Perspective?
• Biological: age, comorbidities, neutropenia and thrombocytopenia, performance status, staging
• Not addressed in case, but also important:
– Psychological: What is the state of their mental health? Do they have adequate coping skills? How likely are they to be able to adhere to various treatment regimens?
– Social: What is their QOL like now? What kind of caregiver support do they have? What kind of financial support might they need for various treatments?
Communication
• Education
• Trust
• Collaboration
• Mutual goals
• Ongoing conversations
• Biological, psychological, and social support
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Tying It All Together
• Reinforce patient and caregiver teaching with each visit
• Identify biological, psychological, and social factors that play into patient’s care and plan
• Ensure patient goals are in line with therapy prescribed
• Encourage questions to promote ongoing education and conversation
Summary
• Nurses, nurse practitioners, and social workers are in the unique role of addressing the multifaceted experience of being treated for MDS
• Education is a key responsibility and piece of the relationship
• Management of side effects, both from disease and treatment, is a priority
• Assess the “whole person” in order to fully address patient needs from diagnosis and throughout treatment
• Ongoing communication re: treatment and treatment goals with patients and caregivers
• Serve as a liaison to other members of the multidisciplinary team to ensure holistic approach to care
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RESOURCES FOR YOU & YOUR PATIENTS
FROM THE LEUKEMIA & LYMPHOMA SOCIETY (LLS)
WWW.LLS.ORG
LLS RESOURCES FOR HEALTHCARE PROFESSIONALS
Online and in-person CE/CME webinars, symposia & rounds
Free CME & CE www.LLS.org/CE
Podcast series for healthcare professionals
Conversations with experts about diagnosing &
treating blood cancers www.LLS.org/HCPpodcast
HCP palm card – User friendly links to resources for you & your patients
www.LLS.org/CE
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LLS RESOURCES FOR PATIENTS AND CAREGIVERS
❑ Information Specialists – disease information, emotional support, financial, travel & co-pay
assistance, local support through LLS patient access field team. Also send free materials to
patients & HCPs.
❑ Nutrition Consultations – One-on-one consultations from certified dietitian
Specialists can serve as a resource for your HCP team
M - F, 9 am to 9 pm ET:
❑ Phone: (800) 955-4572
❑ Live chat: www.LLS.org/InformationSpecialists
❑ Email: [email protected]
❑ Additional support for patients & caregivers – www.LLS.org/Support
❑ Booklets on disease, treatment, & support - www.LLS.org/Booklets
❑ Webinars, videos, in-person programs - www.LLS.org/Programs &
www.LLS.org/Educationvideos
CLINICAL TRIAL NURSE NAVIGATORS
Help patients find and enroll in
clinical trials based on highly
detailed individualized assessments
www.LLS.org/Navigation
602patients provided with in-depth clinical trial
navigation and support in past year
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We have one goal: A world without blood cancers
THANK YOU
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