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HEMATOLOGY > Myelodysplastic Syndromes > Flashcards

Myelodysplastic Syndromes Flashcards

1
Q

In myelodysplastic syndrome, the findings were …

A

heterogeneous and affected all cell lines

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2
Q

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

A

refractory anemia
smoldering leukemia,
oligoblastic leukemia
preleukemia

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3
Q

In 1982, the FAB described the disease known as

A

myelodysplastic syndromes (MDS)

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4
Q

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

A

1982

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5
Q

In 1997, WHO proposed a new classification that included

A

molecular
cytogenetic
immunologic criteria in addition to morphologic features

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6
Q

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

A

myelodysplastic syndrome

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7
Q

certain subtypes of MDS have an increased risk of transforming into

A

acute myeloid leukemia (AML)

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8
Q

MDS median age of diagnosis is

A

76 years old

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9
Q

what cells are affected in MDS

A

erythroid
myeloid
megakaryocytic cells

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10
Q

it was previously believed as the cell of origin for MDS

A

myeloid progenitor cells

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11
Q

It is the true cell of origin of MDS

A

hematopoietic stem cells (HSCs)

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12
Q

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

A

clonal hematopoiesis of indeterminate potential (CHIP)

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13
Q

how many percent of patients older than 65 have CHIP

A

10%

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14
Q

how many percent of patients older than 90 have CHIP

A

20%

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15
Q

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

A

De novo mutations (primary MDS)

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16
Q

a type of MDS that arises as a result of therapy

A

therapy-related MDS (t-MDS)

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17
Q

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

A

chemotherapy or radiotherapy

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18
Q

median onset of t-MDS

A

4-7 years after therapy

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19
Q

examples of cytokines

A

G-CSF
GM-CSF

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20
Q

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

A

t-MDS

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21
Q

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

A

fanconi anemia
diamond-blackfan anemia
shwachman-diamond syndrome

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22
Q

two morphologic findings common in MDS

A

progressive cytopenias
dyspoiesis in one or more cell lines

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23
Q

in early stage apoptosis is

A

increased

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24
Q

in late stage apoptosis is

A

decreased

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25
Q

it is called as the programmed cell death

A

apoptosis

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26
Q

common morphologic findings in dyserythropoiesis is

A

oval macrocytes

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27
Q

in MDS there is normal level of

A

vitamin b12 and folate

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28
Q

morphologic evidence of dyserythropoiesis in PBS

A

oval macrocytes
hypochromic microcytes
dimorphic red blood cells population

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29
Q

morphologic evidence of dyserythropoiesis in BM

A

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

30
Q

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

A

dysmyelopoiesis

31
Q

agranular bands can be easily misclassified as

A

monocytes

32
Q

in the bone marrow, dysmyelopoiesis may be represented by

A

nuclear-cytoplasmic asynchrony

33
Q

morphologic evidence of dysmyelopoiesis

A

persistent basophilic cytoplasm
abnormal granulation
abnormal nuclear shapes
uneven cytoplasmic staining

34
Q

these cells reside in the endosteal surface of the bone marrow

A

myeloblasts
promyelocytes

35
Q

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

A

dysmegakaryopoiesis

36
Q

morphologic evidence of dysmegakaryopoiesis in PBS

A

giant platelets
platelets with abnormal granulation
circulating micromegakaryocytes

37
Q

morphologic evidence of dysmegakaryopoiesis in BM

A

large mononuclear megakaryocytes

micromegakaryocytes or micromegakaryoblast

abnormal nuclear shapes of micromegakaryocytes or micromegakaryoblast

38
Q

it is not sufficient evidence for MDS

A

dysplasia

39
Q

cases where it also causes pancytopenia and dysplasia

A

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

40
Q

it can cause reversible myelodysplasia

A

copper deficiency

41
Q

what are the abnormal cellular functions

A

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

42
Q

what are the FAB classifications

A

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)

43
Q

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

A

10%

44
Q

classification of MDS according to WHO in 2016

A

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

45
Q

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

A

MDS-SLD

46
Q

symptoms of MDS-SLD

A

cytopenia
fatigue
shortness of breath
neutropenia
petechiae
bruising
thrombocytopenia

47
Q

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

A

MDS-MLD

48
Q

in MDS-MLD the myeloblast does not contain

A

auer rods

49
Q

a MDS that contain auer rods

A

MDS-EB-2

50
Q

it reflects the influence of mutations SF3B!

A

MDS-RB

51
Q

in MDS-RB what is gene is mutated

A

SF3B1

52
Q

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

A

MDS-RS-SLD

53
Q

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

A

3-10% and 71 years old

54
Q

MDS-EB-1 blast percentage in the BM

A

5-9%

55
Q

MDS-EB-2 blast percentage in the BM

A

10-19%

56
Q

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

A

MDS-with isolated del(5q)

57
Q

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

A

thalidomide analog lenalidomide (revlimid)

58
Q

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

A

MDS-unclassifiable (MDS-U)

59
Q

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

A

childhood myelodysplastic syndrome

60
Q

gene mutated in childhood myelodysplastic syndrome

A

RUNX1
SOS1
GATA2
ANKRD26

61
Q

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

A

MDS/MPN category

62
Q

what are the MDS/MPN classifications

A

chronic myelomonocytic leukemia (CMML)

atypical chronic myeloid leukemia (aCML)

juvenile chronic myelomonocytic leukemia (JMML)

MDS-MPN with ring sideroblast and thrombocytosis

MDS/MPN-U

63
Q

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

A

atypical chronic myeloid leukemia

64
Q

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

A

atypical chronic myeloid leukemia

65
Q

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

A

juvenile myelomonocytic leukemia (JMML)

66
Q

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

A

juvenile myelomonocytic leukemia (JMML)

67
Q

the mutation in JMML affects what pathway

A

RAS/MAPK pathway

68
Q

JMML has a strong association with what congenital disorder?

A

noonan syndrome
neurofibromatosis type 1

69
Q

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

A

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

70
Q

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

A

MDS/MPN, unclassifiable

71
Q

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

A

epigenetics

72
Q
A

HEMATOLOGY (7 decks)

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