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Block 7 Week 1 > Myelodysplastic Syndromes-Usera > Flashcards

Myelodysplastic Syndromes-Usera Flashcards

1
Q

What is this:
a group of clonal hemtopoietic stem cell diseases characterized by one or more peripheral blood cytopenia(s), with dysplasia in one or more myeloid lines.

A

Myelodysplastic syndromes

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2
Q

With myelodysplastic syndromes there is an increased, but not inevitable, risk for developing (blank)

A

acute leukemia

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3
Q

Who typically gets myelodysplastic syndromes?

A

older adults
around 70
male predominance

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4
Q

What does MDS (myelodysplastic syndromes) look like?

A

blood- cytopenia
marrow- cellular
maturation- variable

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5
Q

What does MPD (myeloproliferative disorder) look like?

A

blood-cythemia
marro-cellular
maturation- yes

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6
Q

What does Acute leukemia look like?

A

blood-variable
marrow-cellular
maturation-no

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7
Q

How do you classify myeodysplastic syndrome?

A

cytopenia
degree of dysplasia in peripheral blood and bone marrow
blast count

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8
Q

What are the IPSS thresholds for cytopenias?

A

hemoglobin less than 10g/Dl
ANC less than 1.8 X 10^9 /L
platelets less than 100 X 10^9/L

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9
Q

What are four types of myelodysplastic syndromes?

A

Refractory Anemia (RA)
Refractory Anemia with Ringed Sideroblasts (RARS)
Refractory Anemia with Excess Blasts (RAEB-1)
Refractory Anemia with Excess Blasts (RAEB-2)

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10
Q

In the myelodysplastic syndrom Refractory Anemia, what do you see in the peripheral blood?

A

anemia

no blasts

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11
Q

In the myelodysplastic syndrome Refractory Anemia, what do you see in the bone marrow?

A

Dysplastic erythroids
less than 5% blasts
less then 15% ringed sideroblasts

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12
Q

In the myelodysplastic syndrome Refractory anemia with ringed sideroblasts (RARS), what do you see in the peripheral blood?

A

anemia
dimorphic red cell pop
pappenhemier bodies
no blasts

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13
Q

In the myelodysplastic syndrome Refractory anemia with ringed sideroblasts (RARS), what do you see in the bone marrow?

A

dysplastic erythroids
less than 5% blasts
GREATER than 15% ringed sideroblasts

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14
Q

In the myelodysplastic syndrome Refractory anemia with excess blasts (RAEB), what do you see in the peripheral blood?

A

Bi-or pancytopenia
In RAEB-1=lless than 5% blasts, NO AUER RODS
In RAEB-2=5-19% blasts or Auer rods

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15
Q

In the myelodysplastic syndrome Refractory anemia with excess blasts (RAEB), what do you see in the bone marrow?

A

5-9% blasts, no auer rods RAEB-1
10-19% blasts or auer rods RAEB-2
Dysplasia

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16
Q

In the myelodysplastic syndrome MDS with del (5q)- 5q syndrome, what do you see in the peripheral blood?

A

anemia, normal to increased platelets
less than 5% blasts
no auer rods

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17
Q

In the myelodysplastic syndrome MDS with del (5q)- 5q syndrome, what do you see in the bone marrow?

A

Hypoblated megakaryocytes
less than 5% blasts
No auer rods
Isolated 5q-karotype

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18
Q

WHy do you get myelodysplastic syndromes?

A

abnormal stem cell clone proliferates

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19
Q

Clonal cytogenic abnormalities are present in (Blank) percent of MDS cases

A

50

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20
Q

What are the initiating stimuli of myelodysplastic syndrome?

A

unknwon but involve genetic damage

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21
Q

Primary or de novo MDS occur without known history of chemotherapy or radiation. What are some possible etiologies?

A

benzene exposure
cigarettes
family Hx of heme neoplasms

22
Q

SOme heritble conditions that are associated with increased risk for MDS are (Blank X 3)

A

Fanconi’s anemia
Schwachman-Diamond syndrome
Diamond-Blackfan syndrome

23
Q

What the clinical findings of myelodysplastic syndromes?

A 
fatigue and weakness
palor
ecchymoses
hemorrhage
infection
24
Q

What are the lab findings of the erythrocytes in myelodysplastic syndromes?

A 
Anemia (HgB less than 10)
macroscytosis (MCV greater than 110)
oval macrocytes
dimorphic anemia in RARS
Reticulocytopenia
Anisocytosis, poikilocytosis
Basophilic stippling
Howell-jolly bodies
nucleated RBC
membrane abnormalities
25
Q

(blank) is a medical term meaning that a patient’s red blood cells are of unequal size. This is commonly found in anemia and other blood conditions.

A

Anisocytosis

26
Q

(blank) refers to the presence of poikilocytes in the blood. Poikilocytes are abnormally shaped red blood cells as seen on a blood film in humans and many wild and domestic species of animals, though they are common in some clinically normal small ruminants, particularly goats.

A

Poikilocytosis

27
Q

(blank) aka Punctate basophilia refers to an observation found when observing a blood smear in which erythrocytes display small dots at the periphery. These dots are the visualization of ribosomes and can often be found in the peripheral blood smear, even in some normal individuals.

A

Basophilic stippling

28
Q

(blank) are histopathological findings of basophilic nuclear remnants (clusters of DNA) in circulating erythrocytes. During maturation in the bone marrow late erythroblasts normally expel their nuclei, but in some cases a small portion of DNA remains

A

Howell–Jolly bodies

29
Q

What are the lab findings of the granulocytes in myelodysplastic syndromes?

A 
Leukopenia
Neutropenia
left-shift sometimes present
agranular/hypogranular neutrophils
abnormal granules
pseudo-pelgeroid nuclei (nonsegmented dense looking nuclei)
hypersegmentation
ring nuclei
30
Q

What are the lab findings of the platelets in myelodysplastic syndromes?

A 
Thrombocytopenia
Thrombocytosis
Giant platelets
Hypogranular platelets
Abnormal granulation
Megakaryoblasts
31
Q

What are the lab findings of the bone marrow in myelodysplastic syndromes?

A

variable cellular amounts
<20% myeloblasts
Dysplastic features in all cell lines common
Abnormal localization of immature precursors
Ringed sideroblasts

32
Q

What are these lab findings of MDS with bone marrow dyserythropoiesis?

A 
Megaloblastoid erythropoiesis
Giant erythroid precursors
Multinucleation
Nuclear fragmentation
Abnormal nuclear shape
Nuclear budding
Karyorrhexis
Irregular nuclear or cytoplasmic staining
Vacuolization
Basophilic stippling
Ringed sideroblasts
33
Q

(blank) is the destructive fragmentation of the nucleus of a dying cell whereby its chromatin is distributed irregularly throughout the cytoplasm

A

Karyorrhexis

34
Q

What are these lab findings of MDS with bone marrow dysgranulopoiesis?

A 
Abnormal granulation
Hypogranularity
Agranularity
Abnormal size granules
Absent secondary granules
Hyposegmentation
Hypersegmentation
35
Q

What are these lab findings of MDS with bone marrow dysmegakaryopoiesis?

A 
Increased megakaryocytes
Normal megakaryocytes
Decreased megakaryocytes
Micromegakaryocytes
Hyperchromatic nuclear staining
Nuclear hyposegmentation
Decreased granularity
Giant platelets
36
Q

What will you find in the peripheral blood of myelodysplastic Refractory anemia?

A

Anemia

Macrocytosis (MCV <1% of nucleated cells

37
Q

What will you find in the bone marrow of Refractory Anemia?

A 
Hypercellular
Erythroid hyperplasia
Dyserythropoiesis
Megaloblastoid changes
Ringed sideroblasts <5% of cellularity
38
Q

What will you find in the peripheral blood of myelodysplastic RA with ringed sideroblasts?

A

Anemia

Macrocytosis (MCV <1% of nucleated cells

39
Q

What will you find in the bone marrow of myelodysplastic RA with ringed sideroblasts?

A 
Hypercellular
Erythroid hyperplasia
Dyserythropoiesis
Megaloblastoid changes
Ringed sideroblasts >15%
Blasts <5% of cellularity
40
Q

IF you see sideroblastic anemia dont just to AML because only (blank) percent involve AML

A

1-2%

41
Q

What will you find in the peripheral blood of myelodysplastic RAEB-1?

A 
Cytopenia in two cell lines
Normo- or macrocytic anemia
Oval macrocytes
Reticulocytopenia
Dysgranulopoiesis
Monocytes <5% of nucleated cells
42
Q

What will you find in the bone marrow of myleodysplastic RAEB-1?

A 
Hypercellular
Dyserythropoiesis
Dysgranulopoiesis
Dysmegakaryopoiesis
Ringed sideroblasts present
Blasts 5-9% of cellularity
Prognosis correlates with blast percentage
43
Q

What will you find in the peripheral blood of myelodysplastic RAEB-2?

A 
Cytopenia in two cell lines
Normo- or macrocytic anemia
Oval macrocytes
Reticulocytopenia
Dysgranulopoiesis
Monocytes 5% of nucleated cells
44
Q

What will you find in the bone marrow of myleodysplastic RAEB-2?

A 
Hypercellular
Dyserythropoiesis
Dysgranulopoiesis
Dysmegakaryopoiesis
Auer rods may be present
Blasts 10-19% of cellularity
Higher risk of leukemia
45
Q

What are myelodysplastic syndrome varients?

A

dont fit into subtype, trilineage dysplasia with less than 5% blasts in marrow, survival is poor with worsening dysplasia

46
Q

What is the hierarchical approach to classification of myelodysplastic syndrome varients?

A

AML
RAEB-2
RAEB-1
RA

47
Q

What is therapy related MDS?

A
  • occurs following chemotherapy or radiation therapy for another disease
  • related to duration, amount, an courses of therapy
  • difficult to classify as specific MDS
  • unfavorable prognosis with high risk leukemia
48
Q

What are the cytogenetics of myelodysplastic syndromes?

A

Frequently involve chromosomes 5 and 7
Isolated 5q- syndrome is a specific Dx associated with good prognosis
Complex karyotypes (>3 abnormalities) are associatd with worse prognosis

49
Q

Detection of chromosomal abnormalities in myelodysplastic syndromes improves with increasing (blank)

A

blast count

50
Q

What is the prognosis of myelodysplastic syndromes?

A

Median survival worsens with increasing blast percentage and trilineage dysplasia
Median survival (all types) is < 2 years
Leukemic transformation 10-40%
Scoring systems aid in prognosis

51
Q

What are the therapies for myelodysplatic syndromes?

A 
Supportive care
RBC transfusion for anemia
Platelets for bleeding prophylaxis
Antibiotics for infections, neutropenia
Growth factors (GM-CSF, EPO)
Chemotherapy for poorer prognosis
Bone marrow transplantation
52
Q

Chemotherapy with the hypomethylating agents (blank) and (blank) has been shown to decrease blood transfuion requirements and to retard the progression of MDS to AML

A

5-azacytidine and decitabine

Block 7 Week 1 (10 decks)

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