Myelodysplastic (or Dysmyelopoietic) Syndromes

Question 1

1. Primary (de novo)
2. Treatment-related (2-5 years post chemotherapy)
3. Very rarely, inherited

Answer 1

3 types of diseases for Myelodysplastic syndromes

Question 2

• chromosomes 5 & 7
• Defect in the Myeloid Stem Cell

Answer 2

Myelodysplastic syndrome etiology

Question 3

ineffective clonal hematopoiesis

Answer 3

too few cells

Question 4

morphological dysplasia

Answer 4

ugly cells

Question 5

______ are a major feature of MDS

Answer 5

Cytopenias

Question 6

Hyperproliferation is NOT seen until the disease transforms into ______

Answer 6

leukemia

Question 7

MDS has progressive ____ (all cell lines can be affected)

Answer 7

cytopenia

Question 8

HALLMARK FINDING of MDS in the bone marrow IS MEGALOBLASTOID ________

Answer 8

Dyserythropoiesis

Question 9

Each MDS is pretty much a basic ___ ____ but with increasingly more severe problems

Answer 9

refractory anemia

Question 10

1st in order of increasing severity

Answer 10

Refractory anemia (RA)

Question 11

2nd in order of increasing severity

Answer 11

Refractory anemia with ringed sideroblasts (RARS)

Question 12

3rd in order of increasing severity

Answer 12

Refractory anemia with excess sideroblasts

Question 13

4th in order of increasing severity

Answer 13

Chronic myelomonocytic leukemia (CMML)

Question 14

5th in order of increasing severity

Answer 14

Refractory anemia with excess blasts in transformation (RAEBIT or RAEB-t)

Question 15

Some patients will actually progress through each MDS classification before it becomes ___!

Answer 15

AML

Question 16

1. Refractory Anemia (RA)
2. Refractory anemia with ringed sideroblasts (RARS)
3. Refractory anemia with excess blasts (RAEB)
4. Condition called MDS unclassified (MDS-U)

Answer 16

WHO subgroups of MDS

Question 17

Refractory cytopenia with unilineage dysplasia

Answer 17

Also known as Refractory Anemia

Question 18

Primary finding is anemia with oval macrocytes and nRBCs

Answer 18

Refractory cytopenia with unilineage dysplasia

Question 19

Rare ringed sideroblasts <15% & siderocytes

Answer 19

Refractory cytopenia with unilineage dysplasia

Question 20

Normal B12 and folate normal

Answer 20

General MDS lab findings

Question 21

<5% bone marrow blasts
Megaloblastoid picture

Answer 21

Refractory cytopenia with unilineage dysplasia

Question 22

MORE ringed sideroblasts >15% of nucleated cells in bone marrow

Answer 22

RARS - refractory anemia with ringed sideroblasts

Question 23

dimorphic RBC population

Answer 23

RARS

Question 24

RA with cytopenia in 2 cell lines now & more blasts

Answer 24

RAEB - refractory anemia with excess blasts

Question 25

bone marrow blasts up to 5-20%

Answer 25

RAEB

Question 26

RA, but mostly monos in the bone marrow

Answer 26

CMML - chronic myelomonocytic leukemia

Question 27

leukocytosis instead of leukopenia

Answer 27

CMML

Question 28

Absolute p.b. monocytosis with morphological abnormalities ALWAYS present

Answer 28

CMML

Question 29

WBC count Normal to decreased

Answer 29

RC, RARS, RAEB

Question 30

Dysgranulopoiesis ++

Answer 30

CMML and RAEB

Question 31

Dyserythropoiesis +++

Answer 31

RC and RARS

Question 32

Megaloblastoid bone marrow with bizarre nRBCs

Answer 32

myelodysplastic syndromes MDS

Question 33

Most frequent aneuploidy (abnormalities) found in MDS

Answer 33

Trisomy 8 and monosomy 7

Question 34

Ineffective hematopoiesis and morphological dysplasia

Answer 34

MDS characteristics