AML Cases

Question 1

Uncontrolled clonal proliferation and accumulation of neoplastic hematopoietic precursor cells of myeloid lineage

Answer 1

AML

Question 2

% of myeloblasts in bone marrow to be considered AML

Answer 2

20% myeloblasts in BM (WHO)

or 30% myeloblasts in BM (FAB)

Question 3

Clinical features of AML

Answer 3

pancytopenia
fever, night sweats, chills, malaise, weight loss
extramedullary disease
hyperleukocytosis
Coagulation abnormalities

Question 4

who classification aml

Answer 4

20%
AML with recurrent cytogenetic abnormalities
t(8;21), t(15;17), t(inv16), 11q23
AML with multilineage dysplasia
AML and MDS, therapy-related
AML not otherwise categorized
similar to FAB list
Acute biphenotypic leukemia

Question 5

best prognostic feature aml

Answer 5

cytogenetics

Question 6

favorable risk genetics

Answer 6

Favorable risk: t(8;21), t(16;16), t(15;17)

Question 7

intermediate risk genetics

Answer 7

Intermediate risk (normal karyotype)
NPM1+/Flt3- genotype most favorable

Question 8

unfavorable risk genetics

Answer 8

Unfavorable risk (del 5, del 7, trisomy 8, 11q23, other complex karyotypes)

Question 9

Treatment AML

Answer 9

Induction chemotherapy 7+3
If remission, then consolidation chemo or transplantation
If no remission, then bad prognostic sign, alternative tx.
Then if remission, consider allogenic transplantation.

Question 10

Consolidation chemo

Answer 10

high dose ara-c x 4 cycles

good risk cytogenetics (8:21, inv16, 15:17)
Normal karyotype w/ NPM1+, Flt3-
Intermediate risk cytogenetics w/o matched sibling donor

Question 11

Allogenic stem cell transplantation

Answer 11

Poor risk cytogenetics
Intermediate-risk cytogenetics with matched sibling donor
Extramedullary disease (chloroma)
Ist or 2nd relapse
Donor sources include siblings, children, parents, MUD, umbilical cord blood

Question 12

Autologous stem cell transplantation

Answer 12

No proven benefit over consolidation chemotherapy in 1st CR

Consider for patients w/o an allogeneic donor

Question 13

tx AML >60 yo

Answer 13

Induction chemotherapy with anthracycline (daunorubicin, mitoxantrone, idarubicin) plus cytosine arabinoside. “7 + 3” regimen
If remission obtained (60% remission rate), then consolidation chemotherapy with reduced intensity cytosine arabinoside
Probably no role for allogeneic transplant
Consider observation and supportive care in lieu of induction chemotherapy

Question 14

most common cytogenetics M3 (Acute promyelocytic leukemia)

Answer 14

t(15:17) - creates fusion gene, PML/RAR-alpha

Question 15

poor risk disease M3 APML

Answer 15

t(11:17)

Question 16

Tx APML

Answer 16

Induction therapy with ATRA plus anthracycline-based chemotherapy
Consolidation with 2 courses anthracycline-based chemotherapy
2 years maintenance chemotherapy with ATRA, 6-MP, and methotrexate
Relapse
arsenic trioxide

Question 17

Common presentation in M3 APML

Answer 17

DIC = disseminated intravascular coagulation

if M3 and DIC = start ATRA in

Question 18

New treatment paradigms for AML

Answer 18

Targeted therapies:
Flt-3 receptor TKIs
CBF