AML

Question 1

The Cancer Genome Atlas (TCGA) and other databases demonstrate that blood cells from up to 5–6% of normal individuals aged >70 years contain potentially “premalignant” mutations that are associated with clonal expansion

Answer 1

clonal hematopoiesis of indeterminate potential (CHIP; sometimes called age-related clonal hematopoiesis)

DNMT3A, TET2, and ASXL1

Question 2

GERMLINE MUTATIONS ASSOCIATED WITH ↑ RISK OF DEVELOPING A MYELOID NEOPLASM

Answer 2

CEBPA, DDX41, RUNX1, ANKRD26, ETV6, and GATA2

Question 3

Required to establish the diagnosis of AML

Answer 3

marrow (or blood) blast count of ≥20%

EXCEPT for AML with the recurrent genetic abnormalities t(15;17), t(8;21), inv(16), or t(16;16)

Question 4

The leading cause of therapy-associated AML

Answer 4

ANTICANCER DRUGS

Question 5

AML with VERY GOOD PROGNOSIS (∼85% cured)

Answer 5

t(15;17)

Question 6

AML with GOOD PROGNOSIS (∼55% cured)

Answer 6

t(8;21) and inv(16)

Question 7

AML with VERY POOR PROGNOSIS

Answer 7

TP53 mutation
complex karyotype

Question 8

One of the most important risk factors

Answer 8

AGE AT DIAGNOSIS

Question 9

Clinical feature associated with a LOWER complete remission (CR) rate and SHORTER survival time

Answer 9

CYTOPENIA

Question 10

Can cause early central nervous system bleeding and pulmonary leukostasis contribute to poor outcomes

Answer 10

Hyperleukocytosis (>100,000/μL)

Question 11

Definition of COMPLETE REMISSION

Answer 11

blood neutrophil count must be ≥1000/μL

platelet count ≥100,000/μL

ABSENT circulating blasts

bone marrow should contain <5% blasts, and ABSENT Auer rods

ABSENT extramedullary leukemia

Question 12

Frequent first symptom among AML patients

Answer 12

FATIGUE

Question 13

Most commonly found in APL

Answer 13

HEMORRHAGIC COMPLICATIONS

APL patients often present with DIC-associated minor hemorrhage but may have significant gastrointestinal bleeding, intrapulmonary hemorrhage, or intracranial hemorrhage

thrombosis - another less frequent but well recognized clinical feature of DIC in APL

Question 14

Median presenting leukocyte count in AML

Answer 14

∼15,000/μL

Question 15

ABNORMAL ROD-shaped granules

Answer 15

AUER RODS

not uniformly present but when they are AML is virtually certain

Question 16

Found at diagnosis in ∼75% of patients

Answer 16

platelet counts <100,000/μL

Question 17

Most commonly used induction regimens (for patients other than those with APL)

Answer 17

combination chemotherapy with cytarabine and an anthracycline (e.g., daunorubicin, idarubicin)

Question 18

Cell cycle S-phase–specific antimetabolite that becomes phosphorylated intracellularly to an active triphosphate form that interferes with DNA synthesis

Answer 18

CYTARABINE

standard dose (100–200 mg/m2) - administered as a continuous intravenous infusion for 7 days

Question 19

DNA intercalators inhibition of topoisomerase II – DNA breaks

Answer 19

Anthracyclines

consists of daunorubicin (60–90 mg/m2) or idarubicin (12 mg/m2) intravenously on days 1, 2, and 3 (the 7 and 3 regimen)

Question 20

May be added to induction therapy for subsets of patients, especially those with CBF AML

Answer 20

CD33-targeting immunoconjugate gemtuzumab ozogamicin

Question 21

Designed to eradicate residual (typically undetectable) leukemic cells to prevent relapse and prolong survival

Answer 21

POSTREMISSION

Question 22

Postremission therapy for younger patient receiving chemotherapy

Answer 22

intermediate- or high-dose cytarabine for 2 – 4 cycles

higher doses of cytarabine during postremission therapy appear more effective than standard doses
(such as are used in induction) for those who do not have adverse risk genetics

Question 23

Best relapse prevention strategy currently available for AML

Answer 23

Allogeneic HCT

Question 24

Platelet transfusions should be given in AML as needed to maintain a platelet count

Answer 24

≥10,000/μL

Question 25

**Remain the major cause of morbidity and death during induction and postremission chemotherapy for AML**

Answer 25

**INFECTIOUS complications**

Question 26

**Acceptable regimens for empiric antibiotic therapy in patients with AML**

Answer 26

**imipenem-cilastatin, meropenem, piperacillin/tazobactam, or an extended-spectrum antipseudomonal cephalosporin (cefepime or ceftazidime)**

Question 27

**Added in neutropenic with catheter-related infections, blood cultures positive for gram-positive bacteria before final identification and susceptibility testing hypotension or shock, or known colonization with penicillin/ cephalosporin-resistant pneumococci or methicillin-resistant Staphylococcus aureus**

Answer 27

**Vancomycin**

Question 28

**Should be considered for antifungal treatment if fever persists for 4–7 days following initiation of empiric antibiotic therapy**

Answer 28

**caspofungin (or a similar echinocandin), voriconazole, isavuconazonium, or liposomal amphotericin B**

Question 29

**An oral drug that induces the differentiation of leukemic cells bearing the t(15;17) where disruption of the RARA gene encoding a retinoid acid receptor occurs**

Answer 29

**TRETINOIN (all-trans-retinoic acid [ATRA])**

Question 30

**SE of ATRA treatment occurring within the first 3 weeks of treatment**

Answer 30

**APL (differentiation) syndrome** ## Footnote characterized by **fever, fluid retention, dyspnea, chest pain, pulmonary infiltrates, pleural and pericardial effusions, and hypoxemia**

Question 31

**Now considered standard for postremission monitoring of APL, at least in high-risk patients**

Answer 31

**sequential monitoring of RT-PCR for PML-RARA**

Question 32

**CONSTANT feature of AML**

Answer 32

**ANEMIA**