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Hematology 2 Lec > MDS PART 2 > Flashcards

MDS PART 2 Flashcards

1
Q

CMPD (Chronic Myeloproliferative Disorders) -Recognized by WHO

A

1) Chronic Myeloid Leukemia
2) Chronic Neutrophilic Leukemia
3) Polycythemia vera
4) Primary myelofibrosis
5) Essential thrombocythemia
6) MPN with eosinophilia (and abnormalities of PDGFRA, PDGFRB, FGFRI)
7) Chronic eosinophilic leukemia
8) Mastocytosis
9) Myeloproliferative neoplasm, unclassifiable

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2
Q

▪ erythrocytic lineage:

A

Refractory Cytopenia with Unilineage Dysplasia (RCUD)

Refractory anemia (RA)

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3
Q

▪ myeloid lineage:

A

Refractory Cytopenia with Unilineage Dysplasia (RCUD)

Refractory neutropenia (RN) – low neut

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4
Q

▪ megakaryocytic lineage:

A

Refractory Cytopenia with Unilineage Dysplasia (RCUD)

Refractory thrombocytopenia

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5
Q

Prognosis: > 5 years AML

A

Refractory Cytopenia with Unilineage Dysplasia (RCUD)

Refractory anemia with ring sideroblasts (RARS)

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6
Q

Progression: 6%

A

Refractory Cytopenia with Unilineage Dysplasia (RCUD)

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7
Q
  • Recent name: MDS with single lineage dysplasia (MDS-SLD)
A

Refractory Cytopenia with Unilineage Dysplasia (RCUD)

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8
Q
  • Not usually progressive to AML
A

Refractory Cytopenia with Unilineage Dysplasia (RCUD)

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9
Q

▪ Unicytopeni a

A

Refractory Cytopenia with Unilineage Dysplasia (RCUD)

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10
Q

▪ No or rare blasts (<1%)

A

Refractory Cytopenia with Unilineage Dysplasia (RCUD)

Refractory cytopenia with multilineage dysplasia (RCMD)

MDS associated with isolated del(5q) or 5q Syndrome

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11
Q

1 cell line was dysplastic

A

Refractory Cytopenia with Unilineage Dysplasia (RCUD)

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12
Q

▪ Unilineage dysplasia

A

Refractory Cytopenia with Unilineage Dysplasia (RCUD)

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13
Q

▪ ≥ 10% of cells in one myeloid lineage (affected)

A

Refractory Cytopenia with Unilineage Dysplasia (RCUD)

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14
Q

▪ < 5% blasts BM

A

Refractory Cytopenia with Unilineage Dysplasia (RCUD)

Refractory anemia with ring sideroblasts (RARS)

Refractory cytopenia with multilineage dysplasia (RCMD)

MDS associated with isolated del(5q) or 5q Syndrome

Myelodysplastic syndrome, unclassified (MDS-U)

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15
Q

▪ (RA): < 15% ringed sideroblasts (rbc surrounded by iron)

A

Refractory Cytopenia with Unilineage Dysplasia (RCUD)

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16
Q

normal WBC and RBC but ↓counts

A

Refractory Cytopenia with Unilineage Dysplasia (RCUD)

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17
Q

none to low (6%) chance of progressing into AML if left untreated

A

Refractory Cytopenia with Unilineage Dysplasia (RCUD)

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18
Q
  • Recent name: Myelodysplastic Syndrome with Ring Sideroblasts (MDS-RS)
A

Refractory anemia with ring sideroblasts (RARS)

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19
Q
  • There is at least 5% that the cells mutated on the gene SF3B1, w/c is present in > 80% of cases
A

Refractory anemia with ring sideroblasts (RARS)

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20
Q

▪ Anemia (rbc lineage affected)

A

Refractory anemia with ring sideroblasts (RARS)

MDS associated with isolated del(5q) or 5q Syndrome

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21
Q

▪ No blasts

A

Refractory anemia with ring sideroblasts (RARS)

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22
Q

▪ Erythroid dysplasia only (RBC only)

A

Refractory anemia with ring sideroblasts (RARS)

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23
Q

▪ 15% ringed sideroblasts

A

Refractory anemia with ring sideroblasts (RARS)

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24
Q

▪ < 5% blasts PB

A

Refractory anemia with excess blasts 1 (RAEB1)

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25
Q

Progression: < 2%

A

Refractory anemia with ring sideroblasts (RARS)

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26
Q

: one dysplastic lineage (erythroid)

A

MDS-RS-SLD (Single Lineage Dysplasia)

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27
Q

2 PB cytopenia

A

MDS-RS-SLD (Single Lineage Dysplasia)

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28
Q

2 or more lineage dysplastic (erythroid, myeloid)

A

MDS-RS-MLD (Multilineage Dysplasia)

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29
Q

3 or more PB cytopenia (↓WBCs, RBCs, PLTs)

A

MDS-RS-MLD (Multilineage Dysplasia)

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30
Q
  • Single-lineage dysplasia
A

MDS-RS-SLD

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31
Q
  • 1 dysplastic lineage but 2 cells can be cytopenic
A

MDS-RS-SLD

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32
Q
  • Erythroid lineage affected w/ low rbc and wbc
A

MDS-RS-SLD

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33
Q
  • Multi–lineage dysplasia
A

MDS-RS-MLD

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34
Q
  • > 1 dysplastic lineage but 3 cells can be cytopenic
A

MDS-RS-MLD

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35
Q
  • Erythroid and myelocytic lineage affected w/ low plt, rbc and wbc
A

MDS-RS-MLD

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36
Q
  • has similarity with MDS-RS-MLD, except it has marked monocytopenia)
A

Refractory cytopenia with multilineage dysplasia (RCMD)

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37
Q

Recent name: MDSMLD

A

Refractory cytopenia with multilineage dysplasia (RCMD)

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38
Q

Prognosis: 33 mons (2 yrs., 9 mons) (bad)

A

Refractory cytopenia with multilineage dysplasia (RCMD)

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39
Q

Progression: 11% (high)

A

Refractory cytopenia with multilineage dysplasia (RCMD)

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40
Q

▪ 3 Cytopenia(s)

A

Refractory cytopenia with multilineage dysplasia (RCMD)

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41
Q

▪ < 1 x 109 /L monocytes (marked dec)

A

Refractory cytopenia with multilineage dysplasia (RCMD)

Refractory anemia with excess blasts 1 (RAEB1)

Refractory anemia with excess blasts 2 (RAEB2)

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42
Q

▪ No Auer rods

A

Refractory cytopenia with multilineage dysplasia (RCMD)

MDS associated with isolated del(5q) or 5q Syndrome

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43
Q

(rod-shaped fragements; appears in more severe type of anemias)

A

Auer rods

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44
Q

▪ Dysplasia in ≥ 10% of cells in 2 or more myeloid lineages (neutrophil and/or erythroid precursors and/or megakaryocytes

A

Refractory cytopenia with multilineage dysplasia (RCMD)

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45
Q

▪ < 5% blasts in marrow

A

Refractory cytopenia with multilineage dysplasia (RCMD)

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46
Q

▪ ± 15% ring sideroblasts

A

Refractory cytopenia with multilineage dysplasia (RCMD)

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47
Q

– deletion of chromosome 5 (q or long arm)

A

MDS associated with isolated del(5q) or 5q Syndrome

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48
Q

Prognosis: < 9 yrs. (px survival)

A

MDS associated with isolated del(5q) or 5q Syndrome

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49
Q

Progression: rare

A

MDS associated with isolated del(5q) or 5q Syndrome

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50
Q
  • not common, but occurs mostly in older women
A

MDS associated with isolated del(5q) or 5q Syndrome

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51
Q

▪ Usually normal or increased platelet count

A

MDS associated with isolated del(5q) or 5q Syndrome

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52
Q

▪ Normal to increased megakaryocytes with hypolobulated nuclei

A

MDS associated with isolated del(5q) or 5q Syndrome

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53
Q
  • leads to typical dysmegakaryopoiesi s and frequent thrombocytosis
A

MDS associated with isolated del(5q) or 5q Syndrome

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54
Q

▪ Cytopenia(s)

A

Refractory cytopenia with multilineage dysplasia (RCMD)
Refractory anemia with excess blasts 1 (RAEB1)
Refractory anemia with excess blasts 2 (RAEB2)
Myelodysplastic syndrome, unclassified (MDS-U)

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55
Q
  • pathological findings more on the bm than in the pb
A

Myelodysplastic syndrome, unclassified (MDS-U)

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56
Q
  • typical: cytogenetic abnormality (unclassified)
A

Myelodysplastic syndrome, unclassified (MDS-U)

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57
Q

▪ Unequivocal dysplasia in < 10% of cells in one or more myeloid cell lines when accompanied by a cytogenetic abnormality considered as presumptive evidence for a diagnosis of MDS

A

Myelodysplastic syndrome, unclassified (MDS-U)

58
Q

OTHER WHO CLASSIFICATION

59
Q

-refers to subtypes of MDS that lacks specific classifications. necessary

A

MYELODYSPLASTIC SYNDROME, UNCLASSIFIABLE

60
Q
  • more pathologic in the bone than in the circulation
A

MYELODYSPLASTIC SYNDROME, UNCLASSIFIABLE

61
Q

CHILDHOOD MYELODYSPLASTIC SYNDROME aka

A

-refractory cytopenia childhood (WHO)

62
Q
  • DE NOVO MDS
A

CHILDHOOD MYELODYSPLASTIC SYNDROME

63
Q

FRENCH-AMERICAN BRITISH (FAB) CLASSIFICATION

A

Refractory Anemia with excess blasts in transformation (RAEBIT)

64
Q

▪ transition period between RAED-1 and RAED-2

A

Refractory Anemia with excess blasts in transformation (RAEBIT)

65
Q

▪ based on morphological features to define specific immunotypes

A

FRENCH-AMERICAN BRITISH (FAB) CLASSIFICATION

66
Q

• PB: 5% blast

A

Refractory Anemia with excess blasts in transformation (RAEBIT)

67
Q

• BM: 20-30% blast (highest among MDS classification)

A

Refractory Anemia with excess blasts in transformation (RAEBIT)

68
Q

• ± Auer rods

A

Refractory Anemia with excess blasts in transformation (RAEBIT)

Refractory anemia with excess blasts 2 (RAEB2)

69
Q

Recent name: MDS with excess blast 1 (MDSEB1)

A

Refractory anemia with excess blasts 1 (RAEB1)

70
Q

Prognosis: < 2 yrs. (shorter)

A

Refractory anemia with excess blasts 1 (RAEB1)

Refractory anemia with excess blasts 2 (RAEB2)

71
Q

Progression: 25%

A

Refractory anemia with excess blasts 1 (RAEB1)

72
Q
  • w/o ringed sideroblasts unlike RARS
A

Refractory anemia with excess blasts 1 (RAEB1)

73
Q

▪ 1 or more Cytopenia(s)

A

Refractory anemia with excess blasts 1 (RAEB1)

74
Q

▪ Unilineage or multilineage dysplasia

A

Refractory anemia with excess blasts 1 (RAEB1)

Refractory anemia with excess blasts 2 (RAEB2)

75
Q

▪ 5%-9% blasts (higher)

A

Refractory anemia with excess blasts 1 (RAEB1)

76
Q

Recent name: MDS with excess blast 2 (MDSEB2)

A

Refractory anemia with excess blasts 2 (RAEB2)

77
Q

Progression: 33%

A

Refractory anemia with excess blasts 2 (RAEB2)

78
Q

▪ 5%-19% blasts (more blast: pronormoblast, megakaryoblas)

A

Refractory anemia with excess blasts 2 (RAEB2)

79
Q

▪ 10%-19% blasts

A

Refractory anemia with excess blasts 2 (RAEB2)

80
Q

Dysplasia is not sufficient evidence for MDS unless there is

81
Q

Oval macrocyte leading to dyserythropoiesis

A

Vitamin B12 or Folate Deficiency

82
Q

Can cause primary MDS

A

chemotherapeutic drugs

83
Q

▪ based on serological properties and cytochemical staining (reviews chromosome translocations and evidence of dysplasia)

A

WHO Classification, 2008

84
Q

▪ group of disorders that result from clonal abnormalities of hematopoietic pluripotential stem cells

A

MYELODYSPLASTIC SYNDROME (MDS)

85
Q

▪ often described as “Pre-Leukemias” (may lead to AML or ALL)

A

MYELODYSPLASTIC SYNDROME (MDS)

86
Q

Group of acquired clonal hematologic disorders characterized by progressive cytopenia

A

MYELODYSPLASTIC SYNDROME (MDS)

87
Q

→ defective erthroid, myeloid, and megakaryocytic maturation

A

MYELODYSPLASTIC SYNDROME (MDS)

88
Q

From the devt of MDS to occurrence of leukemia, cell line continuously decerases (low to zero)

A

MYELODYSPLASTIC SYNDROME (MDS)

89
Q

characterized by hypercellular maturation of the erythroid cells, granulocytes, and megakaryocytes

A

MYELODYSPLASTIC SYNDROME (MDS)

90
Q

▪ age:

A

> 50 yrs. old (Steininger), > 70 yrs. old (Rodaks), average: 50-70 yrs old

91
Q

▪ peripheral blood: characterized by

A

cytopenias

92
Q

o no rbc, few wbc
o normal: agglutination, abnormal: large gaps
o more on leukopenia (low wbc)

A

cytopenias

93
Q

▪ myeloid cell lines: presence of

94
Q

 abnormal devt of bm cell/tissue tissue

95
Q

myeloid cell lines: [?] is affected by

96
Q

▪ clinical course (s/s-diagnosis-treatment): shorter than [?] (longer; year-transition) and longer than ?

A

CMPD

acute leukemias

97
Q

TEST

A

Chemotherapy or Radiation

98
Q

▪ Low cell ct = circulation needs to provide oxy = bm produces myeloblast/wbc precursor infiltrates/accumulates in the circulation = leukemia

A

Chemotherapy or Radiation

99
Q

: problem in the bm; ineffective erthropoiesis

A
  1. Primary-related
100
Q

: normal bm

A
  1. Therapy-related
101
Q

: exposure to radiation kills other healthy cells as it cannot distinguish rbc from wbc = wbc dies, bm becomes dysplastic

A
  1. Therapy-related cause
102
Q

PRIMARY MDS • Abnormal stem cell results from:

A

A) Environmental exposure

B) Mutations caused by chemical, radiation, viral infection or smoking

C) Genetic inheritance of hematologic disorders

103
Q

 Unintentional acquired radiation

A

A) Environmental exposure

104
Q

 Ex. Benzene, chloroform (toxic)

A

B) Mutations caused by chemical, radiation, viral infection or smoking

105
Q

 Clonal abnormalities in meiosis and mitosis

A

C) Genetic inheritance of hematologic disorders

106
Q

 Least likely; caused by unknown gene

A

C) Genetic inheritance of hematologic disorders

107
Q

PRIMARY MDS
Main cause:

A

**Disruption of “apoptosis” responsible for the ineffective hematopoiesis in MDS.

108
Q

 Occurs after 12 days (wbc)/120 days (rbc)

109
Q

: normal rate is disrupted (increased/faster)

A

Predomination of apoptosis

110
Q

 Dysplasia cause:

A

immature cells released in the bm are eliminated

111
Q

Characteristic: more aggressive and evolve quickly into AML

A

THERAPY-RELATED MDS

112
Q

↑ apoptosis = cytopenia

113
Q

↓ apoptosis = proliferation of abnormal cells leading to malignancy

A

LATE/PROGRESSIVE MDS

114
Q

▪ hallmark of myelodysplastic syndrome

115
Q

: caused by CMPD

A

Hyperplasia

116
Q

: abnormal tissue (shape, arrangement); MDS

117
Q

: infiltrated by abnormal development of tissue (cannot produce normal wbc/rbc = cytopenia)

118
Q

 Abnormal rbc prod

A

DYSERYTHROPOIESIS

119
Q

Indication: Howell-Jolly, Basophilic stippling, Sideroblast. Cabot rings

A

DYSERYTHROPOIESIS

120
Q

: iron cells

A

ring sideroblast

121
Q

DYSERYTHROPOIESIS Indications

122
Q

Vitamin B12/folate deficiency

A

Oval macrocytes]

Dimorphic rbc

123
Q

(two population of rbcs combined; micro+macro)

A

Dimorphic rbc

124
Q

Iron deficiency anemia

A

Hypochromic microcytes

125
Q

(heme gives the color) -pale and small rbc due to incomplete Hb prod caused by low iron

A

Hypochromic microcytes

126
Q

 Abnormal wbc prod

A

DYSMYELOPOIESIS

127
Q

 with hyposegmentation/hypersegmentation

A

DYSMYELOPOIESIS

128
Q

 as wbc matures, basophilia dec = segemented neutrophil

A

DYSMYELOPOIESIS

129
Q

o normal:
o hypo:
o hyper:

A

o normal: 3-4 lobes/segements
o hypo: <3
o hyper: >4

130
Q

 monocyte-line not affected: [?] (w/o dysplasia/cytopenia)

A

monocytic hyperplasia

131
Q

= abnormal cells

A

o neutrophil decreases

132
Q

= normal cells

A

o monocyte increases

133
Q

 Abnormal ptl prod and shedding

A

DYSMEGAKARYOPOIESIS

134
Q

 Megakaryocyte, not plt, is present in the circulation or outside the bm (normally, cytoplasm sheds into platelet, nucleus is engulfed by macrophages)

A

DYSMEGAKARYOPOIESIS

135
Q

Age affected by MDS:

A

50-70 yrs old

136
Q

: hypersegmentation of wbc

A

Dysmyelopoiesis

137
Q

Px has both Vit 12 deficiency w/ ineffective erythropoiesis:

A

Oval macrocytes and Dimorphic rbc

138
Q

: cell line affected by MDS

139
Q

: MDS progresses to leukemia

A

↓ apoptosis

140
Q

Dysmegakaryopoiesis:

A

giant platelets, agranular platelet, hypogranular platelet

141
Q

: decreased iron store

Hematology 2 Lec (29 decks)

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