Myelodysplastic Syndromes

Question 1

In myelodysplastic syndrome, the findings were …

Answer 1

heterogeneous and affected all cell lines

Question 2

In MDS, historically this pattern of abnormalities was referred to as

Answer 2

refractory anemia
smoldering leukemia,
oligoblastic leukemia
preleukemia

Question 3

In 1982, the FAB described the disease known as

Answer 3

myelodysplastic syndromes (MDS)

Question 4

In what year does the FAB describe the disease known as myelodysplastic syndromes (MDS)

Answer 4

1982

Question 5

In 1997, WHO proposed a new classification that included

Answer 5

molecular
cytogenetic
immunologic criteria in addition to morphologic features

Question 6

these are groups of acquired clonal hematologic disorders characterized by progressive cytopenias in the peripheral blood

Answer 6

myelodysplastic syndrome

Question 7

certain subtypes of MDS have an increased risk of transforming into

Answer 7

acute myeloid leukemia (AML)

Question 8

MDS median age of diagnosis is

Answer 8

76 years old

Question 9

what cells are affected in MDS

Answer 9

erythroid
myeloid
megakaryocytic cells

Question 10

it was previously believed as the cell of origin for MDS

Answer 10

myeloid progenitor cells

Question 11

It is the true cell of origin of MDS

Answer 11

hematopoietic stem cells (HSCs)

Question 12

it is a condition where healthy patients have clonal hematopoiesis but do not develop hematologic disorders

Answer 12

clonal hematopoiesis of indeterminate potential (CHIP)

Question 13

how many percent of patients older than 65 have CHIP

Answer 13

10%

Question 14

how many percent of patients older than 90 have CHIP

Answer 14

20%

Question 15

it is a mutation that accounts for the most of the cases of MDS

Answer 15

De novo mutations (primary MDS)

Question 16

a type of MDS that arises as a result of therapy

Answer 16

therapy-related MDS (t-MDS)

Question 17

therapy-related MDS (t-MDS) develops after treatment with

Answer 17

chemotherapy or radiotherapy

Question 18

median onset of t-MDS

Answer 18

4-7 years after therapy

Question 19

examples of cytokines

Answer 19

G-CSF
GM-CSF

Question 20

it is an aggressive type of MDS that may evolve quickly into AML

Answer 20

t-MDS

Question 21

what bone marrow failure syndromes have significantly increased risk for developing MDS

Answer 21

fanconi anemia
diamond-blackfan anemia
shwachman-diamond syndrome

Question 22

two morphologic findings common in MDS

Answer 22

progressive cytopenias
dyspoiesis in one or more cell lines

Question 23

in early stage apoptosis is

Answer 23

increased

Question 24

in late stage apoptosis is

Answer 24

decreased

Question 25

it is called as the programmed cell death

Answer 25

apoptosis

Question 26

common morphologic findings in dyserythropoiesis is

Answer 26

oval macrocytes

Question 27

in MDS there is normal level of

Answer 27

vitamin b12 and folate

Question 28

morphologic evidence of dyserythropoiesis in PBS

Answer 28

oval macrocytes hypochromic microcytes dimorphic red blood cells population

Question 29

morphologic evidence of dyserythropoiesis in BM

Answer 29

RBC precursors with more than one nucleus RBC precursors with abnormal nuclear shapes RBC precursors with uneven cytoplasmic staining Ring sideroblasts

Question 30

is suspected when there is a persistence of basophilia in the cytoplasm

Answer 30

dysmyelopoiesis

Question 31

agranular bands can be easily misclassified as

Answer 31

monocytes

Question 32

in the bone marrow, dysmyelopoiesis may be represented by

Answer 32

nuclear-cytoplasmic asynchrony

Question 33

morphologic evidence of dysmyelopoiesis

Answer 33

persistent basophilic cytoplasm abnormal granulation abnormal nuclear shapes uneven cytoplasmic staining

Question 34

these cells reside in the endosteal surface of the bone marrow

Answer 34

myeloblasts promyelocytes

Question 35

it is where the platelets exhibits dyspoietic morphology in the peripheral blood.

Answer 35

dysmegakaryopoiesis

Question 36

morphologic evidence of dysmegakaryopoiesis in PBS

Answer 36

giant platelets platelets with abnormal granulation circulating micromegakaryocytes

Question 37

morphologic evidence of dysmegakaryopoiesis in BM

Answer 37

large mononuclear megakaryocytes micromegakaryocytes or micromegakaryoblast abnormal nuclear shapes of micromegakaryocytes or micromegakaryoblast

Question 38

it is not sufficient evidence for MDS

Answer 38

dysplasia

Question 39

cases where it also causes pancytopenia and dysplasia

Answer 39

vitamin b12 deficiency folate deficiency fanconi-anemia congenital dyserythropoietic anemia parvovirus b19

Question 40

it can cause reversible myelodysplasia

Answer 40

copper deficiency

Question 41

what are the abnormal cellular functions

Answer 41

granulocytes with decreased adhesion deficient phagocytosis decreased chemotaxis impaired microbicidal capacity decreased myeloperoxidase/alkaline phosphatase short RBC survival decreased response to erythropoietin

Question 42

what are the FAB classifications

Answer 42

refractory anemia (RA) refractory anemia with ring sideroblast (RARS) refractory anemia with excess blast (RAEB) chronic myelomonocytic leukemia (CMML) refractory anemia w/ excess blast in transformation (RAEB-t)

Question 43

requirement percentage of dysplastic cells for the diagnosis of MDS according to WHO

Answer 43

10%

Question 44

classification of MDS according to WHO in 2016

Answer 44

MDS-SLD MDS-MLD MDS-RD MDS-EB MDS with isolated del(5q) MDS-unclassifiable

Question 45

a MDS classification where dysplasia must be present in at least one myeloid lineage

Answer 45

MDS-SLD

Question 46

symptoms of MDS-SLD

Answer 46

cytopenia fatigue shortness of breath neutropenia petechiae bruising thrombocytopenia

Question 47

characterized by one or more cytopenias, dysplasia in two or more myeloid cell lines

Answer 47

MDS-MLD

Question 48

in MDS-MLD the myeloblast does not contain

Answer 48

auer rods

Question 49

a MDS that contain auer rods

Answer 49

MDS-EB-2

Question 50

it reflects the influence of mutations SF3B!

Answer 50

MDS-RB

Question 51

in MDS-RB what is gene is mutated

Answer 51

SF3B1

Question 52

it accounts for 3-10% of all MDS cases with median age of presentation of 71

Answer 52

MDS-RS-SLD

Question 53

MDS-RD-SLD accounts for what percent of all cases and what is the median age of presentation

Answer 53

3-10% and 71 years old

Question 54

MDS-EB-1 blast percentage in the BM

Answer 54

5-9%

Question 55

MDS-EB-2 blast percentage in the BM

Answer 55

10-19%

Question 56

a type of MDS that is the only WHO-recognized with a defining cytogenetic abnormality

Answer 56

MDS-with isolated del(5q)

Question 57

it has proven to be effective in patients with isolated del (5q)

Answer 57

thalidomide analog lenalidomide (revlimid)

Question 58

refers to subtypes of MDS that initially lack the specific changes necessary for classification into other MDS subtypes

Answer 58

MDS-unclassifiable (MDS-U)

Question 59

a subtype of MDS where patients have an increased frequency of specific inherited gene mutations such as RUNX1, SOS1, GATA2, ANKRD26

Answer 59

childhood myelodysplastic syndrome

Question 60

gene mutated in childhood myelodysplastic syndrome

Answer 60

RUNX1 SOS1 GATA2 ANKRD26

Question 61

it is a category where there is a presence of characteristics found in MDS/MPN

Answer 61

MDS/MPN category

Question 62

what are the MDS/MPN classifications

Answer 62

chronic myelomonocytic leukemia (CMML) atypical chronic myeloid leukemia (aCML) juvenile chronic myelomonocytic leukemia (JMML) MDS-MPN with ring sideroblast and thrombocytosis MDS/MPN-U

Question 63

it is where the BCR/ABL1 fusion gene is not present

Answer 63

atypical chronic myeloid leukemia

Question 64

it exhibits pelger-huet-like cells, hypogranularity, and bizarre segmentation

Answer 64

atypical chronic myeloid leukemia

Question 65

a clonal disorder characterized by the proliferation of the granulocytic and monocytic cell lines

Answer 65

juvenile myelomonocytic leukemia (JMML)

Question 66

it is a clonal disorder that affects 1 month - 14 years of age

Answer 66

juvenile myelomonocytic leukemia (JMML)

Question 67

the mutation in JMML affects what pathway

Answer 67

RAS/MAPK pathway

Question 68

JMML has a strong association with what congenital disorder?

Answer 68

noonan syndrome neurofibromatosis type 1

Question 69

it is a clonal disorder often associated with a mutation in SF3B1 and JAK2 V617F

Answer 69

MDS/MPN with ring sideroblasts and thrombocytosis (MDS/MPN-RS-T)

Question 70

classification is used for cases that meet the criteria for MDS/MPN but do not fit into one of the aforementioned subcategories

Answer 70

MDS/MPN, unclassifiable

Question 71

it is a term that describes changes in gene expression that occur without altering the DNA sequence

Answer 71

epigenetics