Myelodysplastic Syndrome Flashcards
what does the term senescence refer to?
with age hematopoietic stem cell (HSC) population has a reduced capacity for self-renewal
Hematopoietic stem cell (HSC):
Become more clonal in nature as one ages
and have a predisposition toward?
myeloid cells
risk factors associate with increased risk of MDS can referred to as Therapy-related myeloid neoplasms (TR-MN) which can be further divided into?
therapy-related MDS or AML
patients who receive intensive chemotherapy regimens have an _________ risk of TR-MNs
increased
the International Prognostic Scoring System (IPSS) classification and prognostic assessment includes what genes?
5q or 20q deletions and chromosome 7 abnormalities
what is recognized as a distinct subtype of MDS and has a favorable prognosis?
MDS with 5q deletions
MDS with 5q deletions have a high likelihood of response to what drug?
lenalidomide
for the classification and prognostic factors the multivariate analyses identified four prognostic factors which are?
- cytogenetic abnormalities
- percentage of bone marrow blasts
- age
- number of cytopenias
what are the labs associated with MDS?
anemia
neutropenia
thrombocytopenia
multiple cytopenias
what are the 3 goals of therapy for MDS?
- change natural history of the disease (e.g. delay disease progression)
- reduce number of red blood cell transfusions
- improve quality of life
what are the IMiDs used in lower-risk MDS?
thalidomide (limited b/c intolerable side effects)
linalidomide
IMiDs selectively suppress which del clones by inducing ubiquitination of haplodeficient casein kinase 1A1 (CK1α)?
del(5q)
what are the side effects/toxicities associated with thalidomide?
- fluid retention
- peripheral neuropathy
- thrombosis
- constipation
what level can determine if a patient is a candidate for erythropoiesis-stimulating agents (ESAs)?
EPO level
Patents with lower-risk MDS and symptomatic anemia who have a serum EPO level ____ mU/mL (U/L) can receive growth factor
≤500
what are the growth-colony stimulating factors (GCSF) used in lower-risk MDS?
filgrastim
luspatercept
eltrombopag
which GCSF is a novel erythroid maturation drug?
luspatercept
which GCSF is a novel drugs that stimulate the thrombopoietin receptor similar to endogenous thrombopoietin
eltrombopag
what is the treatment goal of lower-risk MDS patients?
hematologic improvement
immunosuppressive therapy is for what aged patients?
<60
what are immunosuppressive agents that modulate effect T-cells?
antithymocyte globulin (ATG)
cyclosporine
corticosteroids
what are the hypomethylating agents used in lower-risk MDS?
azacitidine
decitabine
what are nucleoside analogs structurally similar to cytosine and capable of being incorporated into DNA instead of cytosine?
hypomethylating agents
what are the side effects/toxicities associated with hypomethylating agents?
- myelosuppression
- febrile neutropenia
- n/v
what is the treatment goal for higher-risk MDS?
change natural history of MDS
what are the 3 treatment options for higher-risk MDS?
DNA hypomethylating agents
allogenic HSCT
IMiDs
what are the hypomethylating agents used for higher-risk MDS?
azacitidine
decitabine with cedazuridine
what is a inhibitor of cytidine deaminase that is used with decitabine to increase decitabine exposure with the oral route?
cedazuridine
what is an oral B-cell lymphoma (BCL-2) inhibitor used with azacitadine?
venetoclax
what is the intensive chemotherapy with AML-type induction combination chemotherapy regimen
anthracycline plus cytarabine
is HSCT recommended for lower-risk MDS?
no
MDS patients are at risk for?
anemia
infections
if patients develop anemia they can have RBC transfusions this cant result in what complication?
iron overload
MDS patients at risk for infections can receive what 2 drugs?
filgrastim and sargramostim
Which of the following is true about complications of myelodysplastic syndrome (MDS)?
a) Patients with myelodysplastic syndrome (MDS) do not have risk of infections
b) Patients with myelodysplastic syndrome (MDS) can be treated with transfusions of red blood cells (RBC) for anemia but complications of transfusions include decrease in iron levels
c) Patients with myelodysplastic syndrome (MDS) can be treated with transfusions of red blood cells (RBC) for anemia but complications of transfusions include iron overload
d) Patients with myelodysplastic syndrome (MDS) do not have any complications due to the disease
C
Patients with myelodysplastic syndrome (MDS) with 5q deletions generally have which of the following?
a) Favorable prognosis and are less likely to respond to lenalidomide
b) Poor prognosis and are less likely to respond to lenalidomide
c) Favorable prognosis and are highly likely to respond to lenalidomide
d) Favorable prognosis and are highly likely to respond to cisplatin
C
Patients with myelodysplastic syndrome (MDS) can receive intensive chemotherapy as a bridge therapy to allogeneic hematopoietic stem cell transplantation (HCST) to?
a) Reduce tumor burden and control disease while suitable donor is found
b) Reduce tumor burden but not to control disease while suitable donor is found
c) Intensive chemotherapy is never given to patients with myelodysplastic syndrome (MDS)
d) Patients with myelodysplastic syndrome (MDS) can only receive targeted therapies for the treatment of the disease
A
