Myelodysplastic syndrome

Myelodysplastic syndrome

MDS

Which hypomethylating agent has phase 3 data showing an improvement in OS ?

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Azacitidine

MDS
MDS

What were the results of the SAKK 33/99 trial ?

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6m improvement in hematologic RR but no change in transformation free survival or OS

MDS
MDS

What did the SAKK33/99 trial assess ?

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ATG + CSA in MDS , phase 3 trial

MDS
MDS

Has the addition of lenalidomide to azacitidine shown to improve ORR or CR ?

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No

MDS
MDS

Has an OS been demonstrated in MDS with the use of decitabine ?

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No, mostly ORR and CR improvements over best supportive care

MDS
MDS

Is there phase 3 data supporting the use of decitabine in MDS ?

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Yes

MDS
MDS

Which phase 3 trials have assessed the superiority of azacitidine in CMML ?

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None

MDS
MDS

Which risk strata patients were utilized in the MEDALIST trial assessing luspatercept in MDS ?

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Very-low, low- and intermediate- risk

MDS
MDS

What were the results of the SUPPORT trial in MDS ?

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The addition of eltrombopag worsened platelet recovery

MDS
MDS

What did the SUPPORT trial assess in MDS ?

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Eltrombopag added to azacitidine

MDS

Which dose and frequency were used in the phase 3 trials showing the efficacy of azacitidine in MDS ?

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75mg/m2 for 7 continuous days

MDS

Which biological pathway does luspatercept target ?

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TGF-Beta

MDS
MDS

What is the most common AE (all grades) seen with luspatercept in MDS ?

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Fatigue

MDS
MDS

What did the MEDALIST trial assess ?

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Luspatercept in very-low, low- and intermediate- risk MDS who were not responding to ESA

MDS
Myelodysplastic syndrome

What percentage ringed sideroblasts are required for a diagnosis of RARS?

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15%

Myelodysplastic syndrome
Myelodysplastic syndrome

What chromosome changes are seen with treatment-related MDS and AML?

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Alkylating agent exposure – del5, del7 – 5-7 year latency period
Topo II inhibitor exposure – 11q23 – 2-3 year latency period

Myelodysplastic syndrome
Myelodysplastic syndrome

Which 2 criteria warrant 7+3 induction for high-risk MDS?

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Blasts % less than or equal to 20%, age less than 60

Myelodysplastic syndrome
Myelodysplastic syndrome

What blast percentage increases the incidence of relapse after an allogeneic stem cell transplant in
MDS?

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5%

Myelodysplastic syndrome
Myelodysplastic syndrome

Which risk strata patients should be considered for allogeneic stem cell transplantation in
myelodysplastic syndrome?

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High risk and very high risk

Myelodysplastic syndrome
Myelodysplastic syndrome

Which 3 cytogenetic changes in myelodysplastic syndrome constitute a diagnosis of AML?

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t(8;21) , inv(16), t(15;17)

Myelodysplastic syndrome
Myelodysplastic syndrome

What are 5 cell surface markers for myeloid blasts?

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CD13,14,33,34,117

Myelodysplastic syndrome
Myelodysplastic syndrome

Which myelodysplastic syndromes present with thrombocytosis?

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5q- and RARS-T

Myelodysplastic syndrome
Myelodysplastic syndrome

Which cytogenetic changes are associated with isolated thrombocytopenia in myelodysplastic
syndrome?

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Deletion 20q

Myelodysplastic syndrome
Myelodysplastic syndrome

What complete blood count changes is most common with myelodysplastic syndrome?

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Anemia

Myelodysplastic syndrome
Myelodysplastic syndrome

What is the relationship between autoimmune disease and myelodysplastic syndrome?

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Increase incidence with polychondritis, polymyalgia rheumatica, Raynaud’s disease, Sjogren’s,
inflammatory bowel disease, pyoderma gangrenosum, glomerulonephritis

Myelodysplastic syndrome
Myelodysplastic syndrome

Which 2 non-malignant hematologic conditions predispose to myelodysplastic syndrome?

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PNH and congenital neutropenia

Myelodysplastic syndrome
Myelodysplastic syndrome

Which 5 genes are commonly mutated in myelodysplastic syndrome?

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DNMT3A, TET2, RUNX1, ASXL4, TP53

Myelodysplastic syndrome
Myelodysplastic syndrome

Which targetable mutations in AML in myelodysplastic syndrome regulate epigenetics?

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IDH1/2

Myelodysplastic syndrome
Myelodysplastic syndrome

Which genes methylate and demethylate DNA in myelodysplastic syndrome?

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DNMT3A- methylation
TET2-demethylation

Myelodysplastic syndrome
Myelodysplastic syndrome

Which genetic mutation is seen in 5q- syndrome ?

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RPS 14

Myelodysplastic syndrome
Myelodysplastic syndrome

Which mutation is seen in sideroblastic MDS? Also seen in RARS-T?

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SF3B1

Myelodysplastic syndrome
Myelodysplastic syndrome

Which cellular organelle is responsible for ringed sideroblast formation?

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Mitochondria

Myelodysplastic syndrome
Myelodysplastic syndrome

What is the median overall survival for patients with high and very high-risk MDS?

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8-18mo

Myelodysplastic syndrome
Myelodysplastic syndrome

Which diagnostic tests are required for patients with myelodysplastic syndrome–myeloproliferative
neoplasm overlap syndrome?

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PDGFRA mutation analysis, to determine if there is a role for imatinib

Myelodysplastic syndrome
Myelodysplastic syndrome

In which group of MDS patients is there a lack of consensus on treatment?

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Intermediate-risk

Myelodysplastic syndrome
Myelodysplastic syndrome

What is the overall response rate for lenalidmoide in 5q- and non-5q- patients?

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67% versus 27%.

Myelodysplastic syndrome
Myelodysplastic syndrome

Which of the hypomethylating agents showed no change in OS?

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Decitabine- Increases ORR, increased Cytogenetics response, but no change in OS

Myelodysplastic syndrome
Myelodysplastic syndrome

Which hypomethylating agent can be given SQ?

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Azacitidine

Myelodysplastic syndrome
Myelodysplastic syndrome

What is the minimum number of months a hypomethylating agent should be used prior to assessing a failure of therapy in MDS?

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4 months

Myelodysplastic syndrome
Myelodysplastic syndrome

Whic variables predict for an elevated response to ATG/CsA in hypocellular MDS?

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age < 60, hypocellular bone marrow, HLA D 12-15 positivity, + PNH clone, Blasts <5%

Myelodysplastic syndrome
Myelodysplastic syndrome

What treatment options are there for low risk and intermediate risk myelodysplastic syndrome with EPO
> 500?

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Hypomethylating agents, IMIDs, immunosuppressive therapy

Myelodysplastic syndrome
Myelodysplastic syndrome

What EPO level should warrant ESA use in MDS?

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Less than 500 mU/mL

Myelodysplastic syndrome
Myelodysplastic Syndrome

What defines a very high risk for myelodysplastic syndrome?

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More than 6 points on the revised international prognostic scoring system

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Which genetic and molecular features are important for stratification of MDS patients?

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TP53 mutation/adverse cytogenetics/IDH mutations

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What is the preferred management approach for patients with MDS patients who harbor a TP53 mutation and are medically fit?

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Allogeneic stem cell transplantation

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Can MDS patients be considered for transplantation after achieving a complete remission with targeted therapy with underlying IDH mutations?

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Yes

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What is the minimum number of cycles that hypomethylating agents should be used in patients with high/very high risk MDS?

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At least 4 cycles

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What management options are available for patients with high risk/very high risk myelodysplastic syndrome who do not achieve a complete remission after intensive chemotherapy?

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Allogeneic stem cell transplant or consolidation chemo

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Which cytogenetic features have poor prognostic implications for patients with MDS?

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Monosomy 7/ 7q deletion

Myelodysplastic Syndrome
Myelodysplastic Syndrome

A minimum of how many chromosomal abnormalities is a harbinger for poor prognosis in patients with MDS?

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3 or more

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Is cytoreductive therapy necessary for high risk/very high risk MDS patients prior to allogeneic stem cell transplantation?

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If the blast count is 10% or higher

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What are cytoreduction options prior to transplant in patients with high risk/very high risk MDS?

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Hypomethylating agents/chemotherapy/targeted therapy in select patients

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Which preoperative regimens are recommended for patients undergoing Allo-HSCT with high risk/very high risk MDS?

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MAC for those under age 65 or RIC/NMA for those over age 65

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Has a survival advantage been demonstrated with hypomethylating agents at high risk/very high risk MDS?

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Yes – azacitidine

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Is there head-to-head comparison between azacitidine and decitabine in high risk MDS?

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No

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Which hypomethylating agent is preferred in patients with an underlying TP53 mutation?

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Decitabine

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Which hypomethylating agent can be administered subcutaneously?

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Azacitidine

Myelodysplastic Syndrome
Myelodysplastic Syndrome

How long should maintenance therapy with hypomethylating agent be continued for patients with high risk/very high risk MDS?

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Indefinitely

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What options are available for patients with high risk/very high risk MDS relapse after allogeneic stem cell transplantation?

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Repeat transplant/donor lymphocyte infusion/hypomethylating agent/clinical trial

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What is a median survival in patients with low risk MDS?

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More than 3 years

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What categorizes patients as low risk MDS?

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IPSS-R 3.0 or less and IPSS 1.0 or less

Myelodysplastic Syndrome
Myelodysplastic Syndrome

In patients with low risk MDS with isolated anemia which biomarker assessment is crucial for determining the treatment pathway?

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EPO with a cutoff of 500 milliunits/mL

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What is the minimum number of weeks EPO should be used in low risk MDS prior to determining a treatment failure?

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12 weeks

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Can myeloid growth factor can assist EPO response in patients with low risk MDS?

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Yes – GM-CSF or G-CSF however there is mixed data

Myelodysplastic Syndrome
Myelodysplastic Syndrome

How long should TPO mimetics be used in low risk MDS with isolated thrombocytopenia prior to determining treatment failure?

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3 months

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Which criteria determine a high response to immune stimulation therapy for low risk MDS?

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Age less than 60/PNH positive clone/STAT3 mutant T-cell clone/ hypocellular bone marrow/maximum 5% blasts in the bone marrow

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What are the management option in low risk MDS patients with isolated anemia and then EPO of more than 500 milliunits/mL?

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Biomarker driven therapy/hypomethylating/lenalidomide agent/decitabine+cedazuridine

Myelodysplastic Syndrome
Myelodysplastic Syndrome

When is Luspatercept appropriate for low risk MDS?

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Ring sideroblasts of at least 15% or 5% with SF3B1 mutation

Myelodysplastic Syndrome
Myelodysplastic Syndrome

When is lenalidomide not an appropriate option in Low risk MDS patients with a 5q deletion?

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In the presence of chromosome 7 abnormalities

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Can lenalidomide be used in low risk MDS without 5q deletions?

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Yes

Myelodysplastic Syndrome
Myelodysplastic Syndrome

If patients do not respond to azacitidine should decitabine be attempted?

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no

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Does lenalidomide delay progression to AML and MDS?

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No

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Is there efficacy of lenalidomide in none 5q deleted MDS?

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Yes – nearly 25% reduction in transfusion requirements

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What are the black box warnings for lenalidomide in the context of MDS?

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Embryo fetal toxicity/severe thrombocytopenia/severe neutropenia/arteriovenous thrombosis/pulmonary embolism

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What is the dosing and frequency recommendation for lenalidomide in low-risk MDS?

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10 mg daily for 21 days on a 28-day cycle

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What is the median duration of response of lenalidomide in 5q deleted MDS?

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2 years

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Which pathway is Luspatercept targeting in MDS?

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SMAD2/3

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What percentage of patients with low-risk MDS can achieve transfusion independence with Luspatercept?

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Approximately 30%

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What are the benefits of utilizing imetelstat in low risk MDS?

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Transfusion independence

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What agents are utilized as part of immunosuppressive therapy for hypocellular MDS?

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ATG and cyclosporine

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Has immunosuppressive therapy been shown to improve survival in MDS?

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No

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What percentage of MDS patients harbor a 5q chromosome deletion?

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Approximately 5%

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What is a classic presentation of 5q deleted MDS?

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Older woman with isolated macrocytic anemia with or without thrombocytosis

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What is the role of eculizumab in hypocellular MDS with a PNH clone?

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Should not be used

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Which MDS patients qualify for Luspatercept?

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Those with at least 15% ringed sideroblasts or 5% with an underlying SF3B1 mutation

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What are the 3 important pathologic hallmarks of poor prognosis for MDS?

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Bone marrow blast percentage/cytogenetic abnormalities/multilineage dysplasia

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Which genetic mutation confers a good prognosis for MDS?

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SF3B1

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Which 4 clinical factors confer worsening prognosis for MDS patients?

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Advancing age/performance status/number of cytopenias/severity of cytopenias

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What are the 3 variables in the IPSS calculator for MDS?

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Bone marrow blast percentage/karyotype/cytopenias

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What are the variables in the IPSS-R prognostic model for MDS ?

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Cytogenetics/bone marrow blast percentage/thrombocytopenia severity/neutropenia severity/anemia severity

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What is the median overall survival in patients with very low risk MDS ?

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9 years

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What indications are there for utilizing a platelet threshold greater than 10k/mcL to administer platelet transfusion in MDS patients?

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Fever/coagulopathy/pulmonary compromise/severe infection and central nervous system events

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What is the long-term concern of thrombopoietin receptor targeting agents in MDS?

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Accelerated transformation to AML

Myelodysplastic Syndrome
Myelodysplastic Syndrome

When should thrombopoietin targeting agents be utilized to manage thrombocytopenia in MDS patients?

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Patients with persistent bleeding despite platelet transfusions and aminocaproic acid

Myelodysplastic Syndrome
Myelodysplastic Syndrome

When should routine prophylactic antimicrobial therapy be used in MDS patients?

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Never

Myelodysplastic Syndrome
Myelodysplastic Syndrome

MDS patients require PRBC transfusions should have a ferritin checked after how many units?

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20

Myelodysplastic Syndrome
Myelodysplastic Syndrome

When should iron chelation therapy be considered in MDS patients?

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those who have passed the 50-unit PRBC mark

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Which packed red blood cell processing techniques are recommended for MDS patients?

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Leukoreduced and irradiated

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What is no longest recommended duration of ESA therapy in MDS patients if there is no proven efficacy?

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24 weeks

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Is there a proven increase in the rate of AML transformation in MDS patients with ESA therapy?

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No

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What evaluation is required in MDS patients prior to starting ESA therapy?

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Thrombotic risk

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Does the dose of ESA correlate with a response rate?

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Yes

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What is the minimum number of weeks ESA should be utilized prior to declaring a treatment failure?

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6 weeks

Myelodysplastic Syndrome
Myelodysplastic Syndrome

What is the median time to detect an ESA response in MDS patients?

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35 days

Myelodysplastic Syndrome
Myelodysplastic Syndrome

Myelodysplastic Syndrome

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Myelodysplastic Syndrome

Myelodysplastic Syndrome
Myelodysplastic Syndrome Part 2

Myelodysplastic Syndrome Part 2

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Myelodysplastic Syndrome Part 2

Myelodysplastic Syndrome Part 2
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