Leukemia

Question 1

The incidence of AML is increased in patients with the following associations?

Answer 1

Prior exposure to radiation, benzene, chemo (especially alkylating agents), prior myeloproliferative disorder, or myelodysplastic syndrome

Question 2

Patients with AML present with what?

Answer 2

Non specific symptoms like fatigue and pallor from anemia, bleeding due to thrombocytopenia and recurrent infections.

Question 3

When the leukocyte is very high in AML what can patients present with?

Answer 3

Leukostasis syndrome which is occlusion of the pulmonary circulation by the blasts causing respiratory failure and cerebral dysfunction.

Question 4

What 2 markers are for mature granulocytic cells?

Answer 4

CD33 and 13

Question 5

Patients with acute promyelocytic leukemia present with what and why?

Answer 5

Significant bleeding because of DIC because of their primary granules

Question 6

What are the 2 most frequent abnormalities in AML de novo?

Answer 6

+8 and -7

Question 7

What is the most common translocation in children, how do we classify it morphologically, what is a high yield association with this translocation, what is the gene involved, and what is the prognosis?

Answer 7

8:21
AML with maturation
Loss of a sex chromosome or 9q22
Runx1
Good in adults, poor in kids.

Question 8

What is the cytogenetic association for acute myelomonocytic leukemia and how do we characterize it?

Answer 8

Translocation 16:16 or inversion 16 (mostly)
AMML with abnormal eosinophils, including large or irregular basophilic granules and positive reactions with periodic acid Schaffer’s and chloroacetate esterase

Question 9

What to remember about treatment response and 16 inversion/translocation?

Answer 9

Very good response to chemo

Question 10

What is the gene involved in the inversion 16, what are the three secondary mutations and which one has a poor prognosis?

Answer 10

CBFB which encodes for part of RUNx1
KIT, KRAS, and NRAS
KIT

Question 11

What leukemia do we see with the 11q translocations, what is the prognosis, and what is the gene?

Answer 11

Acute monocytic leukemia
Poor
KMT2A

Question 12

What is the most common mutation found in AML, what are the most common subtypes we find it in, what is the prognosis?

Answer 12

FLT3 combined with ITD
APL and AML
Poor

Question 13

What subtype do we commonly find NPM1 mutation of AML and what is the prognosis?

Answer 13

Acute monocytic leukemia

It is a good prognosis if it is by itself.

Question 14

What is the first phase of chemo treatment for AML?

Answer 14

Induction where we give a anthracycline for 3 days, a rubicin, and 7 days of continuous infusion of cytarabine.

Question 15

What is the second phase of chemo treatment for AML?

Answer 15

Consolidation

Give high dose cytarabine

Question 16

What is the third phase of chemo treatment for AML?

Answer 16

Maintenance phase

This is where we give ATRA for APL.

Question 17

What is a big time complication for chemo treatment for AML and what do we do about it?

Answer 17

Infections. So patients with neutropenic fever give broad spectrum antibiotics regardless if the cultures are positive until the bone marrow recovers.
Patients with persistent neutropenic fever should get antifungal therapy as well.

Question 18

Wha examination needs to be included in the initial diagnostic evaluation of ALL and why?

Answer 18

Cerebrospinal fluid because there can CNS involvement.

Question 19

What is a big difference between the 9:22 translocation in ALL and CML? Also, what is a mutation highly associated with the 9:22 translocation and what to know about treatment?

Answer 19

The ALL one increases TK activity even more.

IKZF1 gene and are associated with unfavorable outcomes using TKIs

Question 20

Concerning hyperdiploidy, what is the most common chromosome range, above what number of chromosomes is it favorable, what is the most common gained chromosome, which 3 chromosomes gained indicate a good prognosis, and which one if a poor prognosis?

Answer 20

51-60
50
21
4,10,17
5

Question 21

What is the type of leukemia of the translocation 1:19 and what are the markers for the cells?

Answer 21

B cell lineage

10 10 and 9, but negative for 34

Question 22

What is the type of leukemia associated with the 8:14 translocation, what two parts of the body are frequently involved, and what is the prognosis?

Answer 22

Mature B cell all
CNS and abdominal nodal
Poor, but high intensity chemo is making it better

Question 23

What are the 4 drugs to induce treatment for ALL?

Answer 23

Anthracycline, vincristine, L asparaginase and a glucocorticoid.

Question 24

What do we give during the consolidation phase of chemo treatment for ALL?

Answer 24

Multiple agents plus CNS prophylaxis

Question 25

What do we give for the maintenance phase of chemo treatment for ALL?

Answer 25

6 MP and MTX

Question 26

What are the tow drugs we give for APL chemo treatment?

Answer 26

ATRA and arsenic trioxide