MDS Flashcards
What hypomethylating agent has phase 3 date showing an improvement in OS in MDS?
Azacitadine
What biologic pathway does Luspatercept target?
TGF-Beta
Luspatercept Main side effect
Fatigue
What percentage of ringed sideroplasts are required for diagnosis of RARS?
(Refractory Anemia with Ring Sideroblasts)
15%
What chromosome changes are seen with Treatment-related MDS and AML
Alkylating agent exposure
-del 5
-del6
5-7 year latency period
Topo II inhibitor exposure
- 11q23 (2-3 years latency period)
Which 2 criteria warrant 7+3 induction for High risk MDS?
Blasts >20%, age less than 60
What blast percentage increases the incidence of relapse after an allogeneic stem cell transplant in
MDS?
5%
Which risk strata patients should be considered for allogeneic stem cell transplantation in
myelodysplastic syndrome?
High risk and very high risk
Which 3 cytogenetic changes in myelodysplastic syndrome constitute a diagnosis of AML?
t(8;21) , inv(16), t(15;17)
What are 5 cell surface markers for myeloid blasts?
CD13,14,33,34,117
Which myelodysplastic syndromes present with thrombocytosis?
5q- and RARS-T
Which cytogenetic changes are associated with isolated thrombocytopenia in myelodysplastic
syndrome?
Deletion 20q
What complete blood count changes is most common with myelodysplastic syndrome?
Anemia
What is the relationship between autoimmune disease and myelodysplastic syndrome?
Increase incidence with polychondritis, polymyalgia rheumatica, Raynaud’s disease, Sjogren’s,
inflammatory bowel disease, pyoderma gangrenosum, glomerulonephritis
Which 2 non-malignant hematologic conditions predispose to myelodysplastic syndrome?
PNH and congenital neutropenia
Which 5 genes are commonly mutated in myelodysplastic syndrome?
- DNMT3A
- TET2
- RUNX1,
- ASXL4,
- TP53
Which targetable mutations in AML in myelodysplastic syndrome regulate epigenetics?
IDH1/2
Which genes methylate and demethylate DNA in myelodysplastic syndrome?
DNMT3A- methylation
TET2-demethylation
Which genetic mutation is seen in 5q- syndrome ?
RPS 14
Which mutation is seen in sideroblastic MDS? Also seen in RARS-T?
SF3B1
Which cellular organelle is responsible for ringed sideroblast formation?
Mitochondria
What is the median overall survival for patients with high and very high-risk MDS?
8-18 mo
Which diagnostic tests are required for patients with myelodysplastic syndrome–myeloproliferative
neoplasm overlap syndrome?
PDGFRA mutation analysis, to determine if there is a role for imatinib
In which group of MDS patients is there a lack of consensus on treatment?
Intermediate-risk
What is the overall response rate for lenalidmoide in 5q- and non-5q- patients?
67% versus 27%.
Which of the hypomethylating agents showed no change in OS?
Decitabine- Increases ORR, increased Cytogenetics response, but no change in OS
Which hypomethylating agent can be given SQ?
Azacitidine
What is the minimum number of months a hypomethylating agent should be used prior to assessing a failure of therapy in MDS?
4 months
Whic variables predict for an elevated response to ATG/CsA in hypocellular MDS?
age < 60, hypocellular bone marrow, HLA DR 2 and 15 positivity, + PNH clone, Blasts <5%
What treatment options are there for low risk and intermediate risk myelodysplastic syndrome with EPO
> 500?
Hypomethylating agents, IMIDs, immunosuppressive therapy
What EPO level should warrant ESA use in MDS?
Less than 500 mU/mL
MDS with 5q deletion Tx
Lenalidomide
