UNIT 4: MDS Flashcards

1
Q

Group of morphological abnormalities
○ Elderly patients
Heterogenous and affected all cell lines
○ Remain stable for years
○ Progress rapidly to death
Refractory anemia, smoldering leukemia, oligoblastic leukemia, or
preleukemia

A

MDS

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2
Q

Acquired clonal disorders
Progressive cytopenias in PB
○ Defects on all cell lines
○Transform to AML

A

MDS

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3
Q

Progressive cytopenias despite cellular BM
Dyspoiesis in one or more cell lines
Disruption of apoptosis
○ Ineffective hematopoiesis
Transformation to leukemia is apparent
○Apoptosis is decreased

A

MDS

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4
Q

Dyserythropoiesis
Poikilocytosis
Basophilic stippling
Howell-Jolly bodies
Siderocytes

A

MDS

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5
Q

Defective development of RBCs
Observance of dimorphic RBC population
Oval macrocytes, hypochromic microcytes

A

dyserythropoiesis

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6
Q

also observed in cases of vitamin B12
or folic acid deficiency anemia

A

oval macrocytes

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7
Q

determine levels of stored iron,
or the adequacy of stored iron.

A

hypochromic microcytes

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8
Q

Multinucleated
RBC precursor or
abnormal nuclear
shape
Basophilic
stippling
Ringed
sideroblasts

A

dyserythropoiesis

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9
Q

Dimorphic RBC
population
Oval macrocytes
(most common)
Hypochromic
microcytic

A

dyserythropoiesis

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10
Q

N/C asynchrony
Uneven
cytoplasmic
staining
Abnormal
granulation
Abnormal
nuclear
segmentation
Hyperplasia or
hypoplasia

A

dysmyelopoiesis

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11
Q

Basophilia
(indicating
nuclear-cytoplasm
ic asynchrony)
Large granules,
hypogranulation,
or absence of
granules
Hyposegmentation,
hypersegmentatio n, or nuclear rings

A

dysmyelopoiesis

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12
Q

Darkly stained in
both BM and
PBS.

A

dysmyelopoiesis

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13
Q

Large
mononuclear
megakaryocytes
Micromegakaryo cytes or
micromegakaryo blasts
Bilobed or
multiple small
separated nuclei

A

dysmegakaryopoiesis

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14
Q

Dyspoietic
morphology
Giant platelets
Abnormal granulation
hypogranular or agranular
Micromegakaryocytes
maybe present

A

dysmegakaryopoiesis

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15
Q

Dysplasia
○ At least one cell line
Cytogenetic abnormalities
○ 50% of cases
○ None is specific to
diagnosis

A

MDS-SLD

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16
Q

Cytopenias in one or more cell
lines
Dysplasia in two or more cell
lines

17
Q

Formerly known as refractory
cytopenia with multilineage
dysplasia

18
Q

Mutation in SF3B1
○ 5% of ringed nucleated
erythroid cells

19
Q

No mutation detected
○15% of ringed nucleated
erythroid cells

Dimorphic PB
○Hypochromic and
normochromic cells

20
Q

Trilineage cytopenias

21
Q

Auer rods
■ The presence of Auer
rods, regardless of
blast count, qualifies a
case as

22
Q

Affects women predominantly
Anemias without other
cytopenias
No Auer rods

A

MDS ISOLATED DEL (5q)

23
Q

Initially lack specific changes
necessary for classification into
other MDS subtypes
Reclassified when characteristics
develop

24
Q

Increased frequency of specific
inherited mutation
○RUNX1
○SOS1
○GATA2
○ANKRD26

A

childhood MDS

25
the only WHO MDS with a defined cytogenetic abnormality
deleteion of 5q
26
Persistent monocytosis ○ 1.0 x 10⁹/L Absence of BCR/ABL1 Absence of rearrangement of: PDGFRA PCM1-JAK2 ○ PDGFRB ○ FGFR1
CMML
27
BCR/ABL1 Negative Atypical because BCR/ABL1 is negative
ATYPICAL CML
28
Proliferation of granulocytic and monocytic cell lines Children aged 1 month to 14 years Somatic or germline mutation ○ RAS/MAPK pathway Association with congenital disorders such as Noonan syndrome and neurofibromatosis
JMML
29
Dysplasia in one or more Mutation in: ○ TET2 ○ SRSF2 ○ ASXL1 ○ RUNX1 Trisomy 8 ○ loss of all or portions of chromosome 7
CMML
30
Associated with SF3B1 and JAK2V617F mutation
MDS,MPN WITH RING SIDEROBLAST AND THROMBOCYTOSIS
31