AML and MDS

Question 1

acute leukemia

-onset, age, course, immature vs mature

Answer 1

Sudden onset
Can occur in either adults or children
Rapidly fatal without treatment
Composed of immature cells (blasts)

Question 2

chronic leukemia

-onset, age, course, immature vs mature

Answer 2

Slow onset
Occurs only in adults
Longer course
Composed of mature cells

Question 3

malignant proliferation of immature myeloid or lymphoid cells in the bone marrow

Answer 3

acute leukemia

Question 4

acute leukemia cause

Answer 4

Clonal expansion

Maturation failure

Question 5

acute leukemia badness

Answer 5

Crowd out normal cells
Inhibit normal cell function
Infiltrate other organs

Question 6

Clinical findings in acute leukemia

Answer 6

Sudden onset (days)
Symptoms of bone marrow failure
Fatigue
Infections
Bleeding
Bone pain (expanding marrow)
Organ infiltration (liver, spleen, brain)

Question 7

Things have to know about AML

Answer 7

Malignant proliferation of myeloid blasts in blood, bone marrow
20% cutoff for diagnosis
Many subtypes
Bad prognosis

Question 8

how many of the nucleated cells have to be malignant/blast to be diagnosed as AML

Answer 8

20%

Question 9

old classifications of AML - big grouping

Answer 9

M0-3: involve neutrophilic series (myeloblasts, promyelocytes, etc.)
M4-5: involve monocytic series (monoblasts, etc)
M6: involve erythroid series (erythroblasts)
M7: involve megakaryocytic series (megakaryoblasts)

Question 10

dysgranulopoiesis

Answer 10

not enough lobes or cytoplasm in neutrophils

a clue that acute leukemia is probably myeloid

Question 11

how to tell if leukemia is myeloid vs. lymphoid

Answer 11

dysgranulopoiesis
auer rods
cytochemistry (MPO = neutrophils)
immunophenotyping
Cytogenetics

Question 12

Auer rods

Answer 12

azurophilic granules strung together in rods

clue that it is AML

Question 13

Myeloperoxidase shows that cells are from what lineage

Answer 13

neutrophils

Question 14

AML New classification

Answer 14

AML with genetic abnormalities
AML with FLT-3 mutation
AML with multilineage dysplasia
AML, therapy-related
AML, not otherwise classified

Question 15

AML-M0 things you must know

Answer 15

INCREASED myeloblasts
“Bland” blasts
MPO negative
Need markers

Question 16

AML-M1 things you must know

Answer 16

INCREASED myeloblasts
No maturation
Auer rods
MPO positive

Question 17

AML-M2 things you must know

Answer 17

Increased myeloblasts
Maturing neutrophils
t(8;21) in some cases

Question 18

AML-M3 things you must know

Answer 18

INCREASED promyelocytes (special)
Faggot cells
DIC
t(15;17) in all cases

Question 19

Faggot cells

Answer 19

tons of auer rods

M3

Question 20

why is AML-M3 so dangerous

Answer 20

granules stimulate clotting cascade –> clotting and then bleeding = DIC

Question 21

Treatment AML-M3

Answer 21

Use ATRA (retinoic acid) to overcome block in maturation

Question 22

Lab findings in acute leukemia

Answer 22

Blasts/immature cells in blood
Leukocytosis
Anemia
Thrombocytopenia

Question 23

AML-M4 things you must know

Answer 23

increased myeloblasts
increase monocytic cells
extramedullary tumor masses
inv(16) in some cases= good

Question 24

AML-M5 things you must know

Answer 24

Increased monocytic cells
NSE positive
M5A and M5B
Extramedullary tumor masses

Question 25

NSE specific for

Answer 25

monocytic cells

Question 26

AML-M6 things you must know

Answer 26

Inc erythroblasts, inc myeloblasts | dyserythropoiesis

Question 27

AML-M7 things you must know

Answer 27

Incr megakaryoblasts "bland" blasts MPO negative need markers

Question 28

t(8;21)

Answer 28

good prognosis (common in M2)

Question 29

inv(16)

Answer 29

good prognosis (common in M4)

Question 30

t(15;17)

Answer 30

good prognosis (always in M3)

Question 31

11q23

Answer 31

worse prognosis

Question 32

AML with FLT-3 mutation

Answer 32

Mutation of FLT-3 (a tyrosine kinase) Present in 1/3 of cases of AML! Monocytic cells Poor prognosis

Question 33

AML with multilineage dysplasia

Answer 33

Elderly Severe pancytopenia Chromosome abnormalities (5, 7) Poor prognosis

Question 34

AML, therapy-related

Answer 34

Previous chemotherapy 2-5 years to onset Very hard to treat

Question 35

tx AML

Answer 35

chemotherapy | bone marrow transplant

Question 36

prognosis AML

Answer 36

dismal t(8;21), inv(16), t(15;17) better FLT-3, therapy-related worse

Question 37

MDS

Answer 37

Problem: abnormal stem cells Dysmyelopoiesis Maybe inc blasts May evolve into acute leukemia

Question 38

what does dysplasia look like - red cells

Answer 38

megaloblastic nuclei, fragmentation

Question 39

what does dysplasia look like - neutrophils

Answer 39

hypogranulation, hyposegmentation

Question 40

what does dysplasia look like - megakaryocytes

Answer 40

small, non-lobulated cells

Question 41

clinical and lab findings in MDS

Answer 41

older patients asymptomatic, or BM failure macrocytic anemia

Question 42

tx MDS

Answer 42

Low-grade: support, follow. | High-grade: be aggressive.