Myelodysplastic Disorders

Question 1

myelodisplastic syndromes

Answer 1

heterogenous group of clonal hemtaological malignancies characterized by
- PB cytopenias
- Propensity to transform into acute leukemia
- Dysplastic features

Formerly called “preleukemic anemia” or “refractory anemia”

Question 2

Cytopenia - caused by ineffective hematopoiesis

Answer 2

Comes back to our apoptosis balance
- abnormal decrease in apoptosis leads to cell accumulation
- abnormal increase in apoptosis leads to decresed cellular output

In MDS -> there is an abnormal increase in intrameduallary apoptosis in BM
- due to an imbalance between cytokines

Question 3

anti-apoptotic

Answer 3

GM-CSF

Question 4

Apoptotic Increasing

Answer 4

• Fas ligand
• Fas Ag
• TGF-beta
• TNF alpha

Question 5

PB dyserythropoiesis

Answer 5

• internuclear bridging (BM)
• anisocytosis with macrocytosis
• howell jolly body
• basophilic stippling
• teardrop cell

Question 6

BM dyserythropoiesis

Answer 6

• asynchronous development
• nuclear budding
• internuclear bridging
• basophilic stippling

Question 7

dysgranulocytosis PB

Answer 7

• hyposegmentation
• bilobulation (pseudo-Pelger-Huet)
• Hypogranulation

Question 8

dysgranulocytopoitesis BM

Answer 8

hyposegmentation (pseudo-pelger-huet)
hypogranulation/agranulation

Question 9

dysmegakaryocytosis PB

Answer 9

giant platelet

Question 10

dysmegakaryocytopoiesis BM

Answer 10

• megakaryotcytoes with detached nuclei
• hypogranulation

Question 11

MDS phenotype changes as disease progresses toward leukemia

Answer 11

BM
- blasts increase

PB
- Leukopenia changes to leukocytosis

Hepatosplenomegaly can progress
MDS evolves into a proliferative blood disorder

Question 12

MDS affects

Answer 12

predominantly the elderly
- median age 70

Men are effected more

can be seen following a viral infection

can occur in patients treated with chemotherapy and radiation
- secondary MDS

Question 13

MDS genetic abnormnalities

Answer 13

LOSS of genetic matieral
- chromosome deletions involving part of or whole chromosome

This is the opposite of Acute leukemias which are translocations

Question 14

MDS clinical features

Answer 14

BM failure
- anemia
- neutropenia
- thrombocytopenia

Symptoms
- fatigue
- recurrent infections
- bleeding
- fever

Question 15

cytopenia definition

Answer 15

Hgb
PLT
Absolute neutrophil count
monocyte

Question 16

Single Lineage Dysplasia - MDS

Dysplastic lineages
Cytopenias
BM
PB
Genetics

Answer 16

Dysplastic Lineage: 1

Cytopenias: 1-2

BM : <5% Blasts

PB: <1% Blasts

Question 17

Multilineage Dysplasia

Dysplastic lineages
Cytopenias
BM
PB
Genetics

Answer 17

Dysplastic lineages: 2-3
Cytopenias: 1-3
BM: <5% Blasts
PB: <1% Blasts
Genetics

Question 18

Ringed Sideroblasts and single lineage Dysplasia

Answer 18

Dysplastic lineages: 1
Cytopenias: 1-2
BM: <5% blasts, more or equal to 15% Ringed Sideroblasts
PB: <1% Blasts
Genetics

Question 19

Ringed Sideroblasts and multilineage dysplasia

Answer 19

Dysplastic lineages: 2-3
Cytopenias: 1-3
BM: <5% Blasts, more than or equal to 15% ringed sideroblasts
PB: <1% Blasts
Genetics

Question 20

Ringed sideroblasts

Answer 20

> 10 iron granules covering <1/3 nuclear rim

Question 21

Excess Blast 1

Answer 21

Dysplastic lineages: 1-3
Cytopenias: 1-3
BM: 5-9% Blasts
PB: 2-4% blasts
Genetics

Question 22

Excess Blasts 2

Answer 22

Dysplastic lineage: 1-3
Cytopenias: 1-3
BM: 10-19% Blasts or Auer rods
PB: 5-29% Blasts or Auer Rods present

Question 23

Isolated del(5q)

Answer 23

dysplastic lineages: 1-3
Cytopenias: 1-2
BM: <5% blasts
PB: <1% Blasts
Genetics: Sq deletion