MDS PART 2 Flashcards
(141 cards)
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
2
Q
▪ erythrocytic lineage:
A
Refractory Cytopenia with Unilineage Dysplasia (RCUD)
Refractory anemia (RA)
3
Q
▪ myeloid lineage:
A
Refractory Cytopenia with Unilineage Dysplasia (RCUD)
Refractory neutropenia (RN) – low neut
4
Q
▪ megakaryocytic lineage:
A
Refractory Cytopenia with Unilineage Dysplasia (RCUD)
Refractory thrombocytopenia
5
Q
Prognosis: > 5 years AML
A
Refractory Cytopenia with Unilineage Dysplasia (RCUD)
Refractory anemia with ring sideroblasts (RARS)
6
Q
Progression: 6%
A
Refractory Cytopenia with Unilineage Dysplasia (RCUD)
7
Q
- Recent name: MDS with single lineage dysplasia (MDS-SLD)
A
Refractory Cytopenia with Unilineage Dysplasia (RCUD)
8
Q
- Not usually progressive to AML
A
Refractory Cytopenia with Unilineage Dysplasia (RCUD)
9
Q
▪ Unicytopeni a
A
Refractory Cytopenia with Unilineage Dysplasia (RCUD)
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
11
Q
1 cell line was dysplastic
A
Refractory Cytopenia with Unilineage Dysplasia (RCUD)
12
Q
▪ Unilineage dysplasia
A
Refractory Cytopenia with Unilineage Dysplasia (RCUD)
13
Q
▪ ≥ 10% of cells in one myeloid lineage (affected)
A
Refractory Cytopenia with Unilineage Dysplasia (RCUD)
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)
15
Q
▪ (RA): < 15% ringed sideroblasts (rbc surrounded by iron)
A
Refractory Cytopenia with Unilineage Dysplasia (RCUD)
16
Q
normal WBC and RBC but ↓counts
A
Refractory Cytopenia with Unilineage Dysplasia (RCUD)
17
Q
none to low (6%) chance of progressing into AML if left untreated
A
Refractory Cytopenia with Unilineage Dysplasia (RCUD)
18
Q
- Recent name: Myelodysplastic Syndrome with Ring Sideroblasts (MDS-RS)
A
Refractory anemia with ring sideroblasts (RARS)
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)
20
Q
▪ Anemia (rbc lineage affected)
A
Refractory anemia with ring sideroblasts (RARS)
MDS associated with isolated del(5q) or 5q Syndrome
21
Q
▪ No blasts
A
Refractory anemia with ring sideroblasts (RARS)
22
Q
▪ Erythroid dysplasia only (RBC only)
A
Refractory anemia with ring sideroblasts (RARS)
23
Q
▪ 15% ringed sideroblasts
A
Refractory anemia with ring sideroblasts (RARS)
24
Q
▪ < 5% blasts PB
A
Refractory anemia with excess blasts 1 (RAEB1)
25
Progression: < 2%
Refractory anemia with ring sideroblasts (RARS)
26
: one dysplastic lineage (erythroid)
MDS-RS-SLD (Single Lineage Dysplasia)
27
2 PB cytopenia
MDS-RS-SLD (Single Lineage Dysplasia)
28
2 or more lineage dysplastic (erythroid, myeloid)
MDS-RS-MLD (Multilineage Dysplasia)
29
3 or more PB cytopenia (↓WBCs, RBCs, PLTs)
MDS-RS-MLD (Multilineage Dysplasia)
30
- Single-lineage dysplasia
MDS-RS-SLD
31
- 1 dysplastic lineage but 2 cells can be cytopenic
MDS-RS-SLD
32
- Erythroid lineage affected w/ low rbc and wbc
MDS-RS-SLD
33
- Multi--lineage dysplasia
MDS-RS-MLD
34
- >1 dysplastic lineage but 3 cells can be cytopenic
MDS-RS-MLD
35
- Erythroid and myelocytic lineage affected w/ low plt, rbc and wbc
MDS-RS-MLD
36
- has similarity with MDS-RS-MLD, except it has marked monocytopenia)
Refractory cytopenia with multilineage dysplasia (RCMD)
37
Recent name: MDSMLD
Refractory cytopenia with multilineage dysplasia (RCMD)
38
Prognosis: 33 mons (2 yrs., 9 mons) (bad)
Refractory cytopenia with multilineage dysplasia (RCMD)
39
Progression: 11% (high)
Refractory cytopenia with multilineage dysplasia (RCMD)
40
▪ 3 Cytopenia(s)
Refractory cytopenia with multilineage dysplasia (RCMD)
41
▪ < 1 x 109 /L monocytes (marked dec)
Refractory cytopenia with multilineage dysplasia (RCMD)
Refractory anemia with excess blasts 1 (RAEB1)
Refractory anemia with excess blasts 2 (RAEB2)
42
▪ No Auer rods
Refractory cytopenia with multilineage dysplasia (RCMD)
MDS associated with isolated del(5q) or 5q Syndrome
43
(rod-shaped fragements; appears in more severe type of anemias)
Auer rods
44
▪ Dysplasia in ≥ 10% of cells in 2 or more myeloid lineages (neutrophil and/or erythroid precursors and/or megakaryocytes
Refractory cytopenia with multilineage dysplasia (RCMD)
45
▪ < 5% blasts in marrow
Refractory cytopenia with multilineage dysplasia (RCMD)
46
▪ ± 15% ring sideroblasts
Refractory cytopenia with multilineage dysplasia (RCMD)
47
– deletion of chromosome 5 (q or long arm)
MDS associated with isolated del(5q) or 5q Syndrome
48
Prognosis: < 9 yrs. (px survival)
MDS associated with isolated del(5q) or 5q Syndrome
49
Progression: rare
MDS associated with isolated del(5q) or 5q Syndrome
50
- not common, but occurs mostly in older women
MDS associated with isolated del(5q) or 5q Syndrome
51
▪ Usually normal or increased platelet count
MDS associated with isolated del(5q) or 5q Syndrome
52
▪ Normal to increased megakaryocytes with hypolobulated nuclei
MDS associated with isolated del(5q) or 5q Syndrome
53
- leads to typical dysmegakaryopoiesi s and frequent thrombocytosis
MDS associated with isolated del(5q) or 5q Syndrome
54
▪ Cytopenia(s)
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)
55
- pathological findings more on the bm than in the pb
Myelodysplastic syndrome, unclassified (MDS-U)
56
- typical: cytogenetic abnormality (unclassified)
Myelodysplastic syndrome, unclassified (MDS-U)
57
▪ 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
Myelodysplastic syndrome, unclassified (MDS-U)
58
OTHER WHO CLASSIFICATION
59
-refers to subtypes of MDS that lacks specific classifications. necessary
MYELODYSPLASTIC SYNDROME, UNCLASSIFIABLE
60
- more pathologic in the bone than in the circulation
MYELODYSPLASTIC SYNDROME, UNCLASSIFIABLE
61
CHILDHOOD MYELODYSPLASTIC SYNDROME aka
-refractory cytopenia childhood (WHO)
62
- DE NOVO MDS
CHILDHOOD MYELODYSPLASTIC SYNDROME
63
FRENCH-AMERICAN BRITISH (FAB) CLASSIFICATION
Refractory Anemia with excess blasts in transformation (RAEBIT)
64
▪ transition period between RAED-1 and RAED-2
Refractory Anemia with excess blasts in transformation (RAEBIT)
65
▪ based on morphological features to define specific immunotypes
FRENCH-AMERICAN BRITISH (FAB) CLASSIFICATION
66
• PB: 5% blast
Refractory Anemia with excess blasts in transformation (RAEBIT)
67
• BM: 20-30% blast (highest among MDS classification)
Refractory Anemia with excess blasts in transformation (RAEBIT)
68
• ± Auer rods
Refractory Anemia with excess blasts in transformation (RAEBIT)
Refractory anemia with excess blasts 2 (RAEB2)
69
Recent name: MDS with excess blast 1 (MDSEB1)
Refractory anemia with excess blasts 1 (RAEB1)
70
Prognosis: < 2 yrs. (shorter)
Refractory anemia with excess blasts 1 (RAEB1)
Refractory anemia with excess blasts 2 (RAEB2)
71
Progression: 25%
Refractory anemia with excess blasts 1 (RAEB1)
72
- w/o ringed sideroblasts unlike RARS
Refractory anemia with excess blasts 1 (RAEB1)
73
▪ 1 or more Cytopenia(s)
Refractory anemia with excess blasts 1 (RAEB1)
74
▪ Unilineage or multilineage dysplasia
Refractory anemia with excess blasts 1 (RAEB1)
Refractory anemia with excess blasts 2 (RAEB2)
75
▪ 5%-9% blasts (higher)
Refractory anemia with excess blasts 1 (RAEB1)
76
Recent name: MDS with excess blast 2 (MDSEB2)
Refractory anemia with excess blasts 2 (RAEB2)
77
Progression: 33%
Refractory anemia with excess blasts 2 (RAEB2)
78
▪ 5%-19% blasts (more blast: pronormoblast, megakaryoblas)
Refractory anemia with excess blasts 2 (RAEB2)
79
▪ 10%-19% blasts
Refractory anemia with excess blasts 2 (RAEB2)
80
Dysplasia is not sufficient evidence for MDS unless there is
cytopenia
81
Oval macrocyte leading to dyserythropoiesis
Vitamin B12 or Folate Deficiency
82
Can cause primary MDS
chemotherapeutic drugs
83
▪ based on serological properties and cytochemical staining (reviews chromosome translocations and evidence of dysplasia)
WHO Classification, 2008
84
▪ group of disorders that result from clonal abnormalities of hematopoietic pluripotential stem cells
MYELODYSPLASTIC SYNDROME (MDS)
85
▪ often described as "Pre-Leukemias" (may lead to AML or ALL)
MYELODYSPLASTIC SYNDROME (MDS)
86
Group of acquired clonal hematologic disorders characterized by progressive cytopenia
MYELODYSPLASTIC SYNDROME (MDS)
87
→ defective erthroid, myeloid, and megakaryocytic maturation
MYELODYSPLASTIC SYNDROME (MDS)
88
From the devt of MDS to occurrence of leukemia, cell line continuously decerases (low to zero)
MYELODYSPLASTIC SYNDROME (MDS)
89
characterized by hypercellular maturation of the erythroid cells, granulocytes, and megakaryocytes
MYELODYSPLASTIC SYNDROME (MDS)
90
▪ age:
> 50 yrs. old (Steininger), > 70 yrs. old (Rodaks), average: 50-70 yrs old
91
▪ peripheral blood: characterized by
cytopenias
92
o no rbc, few wbc
o normal: agglutination, abnormal: large gaps
o more on leukopenia (low wbc)
cytopenias
93
▪ myeloid cell lines: presence of
dysplasia
94
abnormal devt of bm cell/tissue tissue
dysplasia
95
myeloid cell lines: [?] is affected by
wbc
96
▪ clinical course (s/s-diagnosis-treatment): shorter than [?] (longer; year-transition) and longer than [?](shorter; month-transition)
CMPD
acute leukemias
97
TEST
Chemotherapy or Radiation
98
▪ Low cell ct = circulation needs to provide oxy = bm produces myeloblast/wbc precursor infiltrates/accumulates in the circulation = leukemia
Chemotherapy or Radiation
99
: problem in the bm; ineffective erthropoiesis
1. Primary-related
100
: normal bm
2. Therapy-related
101
: exposure to radiation kills other healthy cells as it cannot distinguish rbc from wbc = wbc dies, bm becomes dysplastic
2. Therapy-related cause
102
PRIMARY MDS • Abnormal stem cell results from:
A) Environmental exposure
B) Mutations caused by chemical, radiation, viral infection or smoking
C) Genetic inheritance of hematologic disorders
103
Unintentional acquired radiation
A) Environmental exposure
104
Ex. Benzene, chloroform (toxic)
B) Mutations caused by chemical, radiation, viral infection or smoking
105
Clonal abnormalities in meiosis and mitosis
C) Genetic inheritance of hematologic disorders
106
Least likely; caused by unknown gene
C) Genetic inheritance of hematologic disorders
107
PRIMARY MDS
Main cause:
**Disruption of "apoptosis" responsible for the ineffective hematopoiesis in MDS.
108
Occurs after 12 days (wbc)/120 days (rbc)
apoptosis
109
: normal rate is disrupted (increased/faster)
Predomination of apoptosis
110
Dysplasia cause:
immature cells released in the bm are eliminated
111
Characteristic: more aggressive and evolve quickly into AML
THERAPY-RELATED MDS
112
↑ apoptosis = cytopenia
EARLY MDS
113
↓ apoptosis = proliferation of abnormal cells leading to malignancy
LATE/PROGRESSIVE MDS
114
▪ hallmark of myelodysplastic syndrome
DYSPLASIA
115
: caused by CMPD
Hyperplasia
116
: abnormal tissue (shape, arrangement); MDS
Dysplasia
117
: infiltrated by abnormal development of tissue (cannot produce normal wbc/rbc = cytopenia)
Cancer
118
Abnormal rbc prod
DYSERYTHROPOIESIS
119
Indication: Howell-Jolly, Basophilic stippling, Sideroblast. Cabot rings
DYSERYTHROPOIESIS
120
: iron cells
ring sideroblast
121
DYSERYTHROPOIESIS Indications
122
Vitamin B12/folate deficiency
Oval macrocytes]
Dimorphic rbc
123
(two population of rbcs combined; micro+macro)
Dimorphic rbc
124
Iron deficiency anemia
Hypochromic microcytes
125
(heme gives the color) -pale and small rbc due to incomplete Hb prod caused by low iron
Hypochromic microcytes
126
Abnormal wbc prod
DYSMYELOPOIESIS
127
with hyposegmentation/hypersegmentation
DYSMYELOPOIESIS
128
as wbc matures, basophilia dec = segemented neutrophil
DYSMYELOPOIESIS
129
o normal:
o hypo:
o hyper:
o normal: 3-4 lobes/segements
o hypo: <3
o hyper: >4
130
monocyte-line not affected: [?] (w/o dysplasia/cytopenia)
monocytic hyperplasia
131
= abnormal cells
o neutrophil decreases
132
= normal cells
o monocyte increases
133
Abnormal ptl prod and shedding
DYSMEGAKARYOPOIESIS
134
Megakaryocyte, not plt, is present in the circulation or outside the bm (normally, cytoplasm sheds into platelet, nucleus is engulfed by macrophages)
DYSMEGAKARYOPOIESIS
135
Age affected by MDS:
50-70 yrs old
136
: hypersegmentation of wbc
Dysmyelopoiesis
137
Px has both Vit 12 deficiency w/ ineffective erythropoiesis:
Oval macrocytes and Dimorphic rbc
138
: cell line affected by MDS
Myeloid
139
: MDS progresses to leukemia
↓ apoptosis
140
Dysmegakaryopoiesis:
giant platelets, agranular platelet, hypogranular platelet
141
: decreased iron store
MPD
