29 Acute Leukemias

Amanda F. Cashen

QUESTIONS

Each of the numbered items below is followed by lettered answers. Select the ONE lettered answer that is BEST in each case unless instructed otherwise.

Question 29.1 The (8;21) cytogenetic rearrange, which is associated with favorable risk in acute myeloid leukemia (AML), juxtaposes which genes?

A. AML1-ETO

B. RUNX1-RUNX1T1

C. A core-binding factor and a zinc finger protein

D. All of the above

Question 29.2 You are caring for a 25-year-old woman with AML-M2 and normal cytogenetics. She receives 7 + 3 induction therapy, and her day-14 bone marrow biopsy is ablated. She returns to clinic and her day-45 bone marrow shows normal hematopoiesis. However, she relapses after 18 months. Which of the following likely contributed to her relapse?

A. DNMT3A mutation

B. CEBPA mutation

C. NPM mutation without FLT3-ITD

D. Her age

Question 29.3 Your well-read 30-year-old patient with AML asks you how you will follow her disease once she is in remission. She asks which is the most sensitive test for minimal residual disease. In which order are these assays most sensitive (least to most sensitive)?

A. Cytopathology < fluorescence in situ hybridization (FISH) < polymerase chain reaction (PCR)

B. FISH < cytogenetics < PCR

C. PCR < cytopathology < FISH

D. Cytogenetics < PCR < flow cytometry

Question 29.4 Mutation of which of the following genes is associated with a worse prognosis in core-binding factor AML?

A. ABL

B. CKIT

C. NPM1

D. GATA-1

Question 29.5 A 52-year-old man presents with AML. On day 2 of induction therapy, he develops diffuse alveolar hemorrhage. An expected physical finding on examination would be:

A. Diffuse ecchymosis.

B. Diffuse intravascular coagulopathy.

C. Swollen gums.

D. Cardiac rub.

Questions 29.6–7 You have been following a 75-year-old woman in clinic with mild renal insufficiency and progressive anemia. She has good performance status and presents to clinic with a 3-day history of progressive fatigue and new headaches. Her white blood cell (WBC) count is 53,000 with 50% blasts.

Question 29.6 Given her history, what is the expected finding in the cytogenetics?

A. t(15;17)

B. –7

C. inv(16)

D. t(8;21)

Question 29.7 Which of the following is associated with improved prognosis in this patient?

A. Probable leukostasis

B. Her age

C. Prior myelodysplastic syndrome (MDS)

D. Good performance status

Question 29.8 Which of the following cytogenetic profiles from an AML patient is an example of monosomal karyotype?

A. −7

B. +8, inv(3), del(5q)

C. inv(16)

D. −7, −5, t(3;21)

Question 29.9 Which of the following is a poor prognostic factor in adult ALL?

A. Age <55 years

B. WBC <10,000

C. Diploid chromosomes on karyotyping

D. t(4;11)

Question 29.10 A 70-year-old man presents with newly diagnosed AML-M4 with eosinophilia. Which of the following factors will have the greatest impact on the choice of therapy?

A. His age

B. His performance status

C. His cytogenetics

D. Excellent response to 7 + 3 induction therapy in the elderly

Question 29.11 You have been following a 54-year-old woman with acute promyelocytic leukemia (APL) in clinic. After 2 years she returns to clinic with fatigue, an elevated WBC, and increased promyelocytes. Peripheral blood PCR confirms recurrence of her t(15;17) translocation. Before starting arsenic salvage therapy, which test you obtain?

A. Liver function test

B. Erythrocyte sedimentation rate

C. D-dimer

D. Electrocardiogram

Question 29.12 You have been caring for a 65-year-old woman with a distant history of breast cancer treated with adjuvant cyclophosphamide and adriamycin. During the last year, she developed progressive anemia and thrombocytopenia. She also recently developed leukopenia. Her bone marrow biopsy shows decreased cellularity with dysplastic features and 25% blasts. Which of the following cytogenetic changes might you expect to find?

A. t(15;17)

B. t(9;21)

C. Complex cytogenetics

D. Trisomy 21

Question 29.13 Which of the following statements is TRUE regarding elderly patients (age >65 years) with AML? (Select two correct responses)

A. In general, older patients with AML have poor outcomes when compared to younger patients.

B. Treatment with hypomethylating agents leads to the same response rate as more intensive chemotherapy.

C. Older patients are more likely to have favorable risk cytogenetics than younger patients.

D. Treatment with decitabine leads to better response rates but similar survival compared to low-dose cytarabine.

Questions 29.14–18 You are consulted to see a 20-year-old Hispanic woman who presented with progressive fatigue during the last week and then significant epistaxis. Her WBC is 12,000/μL with 40% promyelocytes and a platelet count of 15,000/μL. Her international normalized ratio (INR) is 2.7 with a prothrombin time of 45 and partial thromboplastin time of 65. Her fibrinogen is 82. On review of her peripheral smear, you observe many promyelocytes with large granules and multiple Auer rods.

Question 29.14 After review of her peripheral smear, you suspect that she has APL. While awaiting confirmation of the diagnosis, your initial therapy should include which of the following?

A. Steroids

B. Cytarabine

C. Fresh-frozen plasma

D. Arsenic trioxide

Question 29.15 Three days after starting idarubicin and ATRA, her coagulopathy has improved. She has shortness of breath in the morning and rapidly becomes hypoxic over the course of the day. Which of the following would prove most helpful in treating her hypoxia?

A. High-flow facemask oxygen

B. Lasix

C. Methylprednisolone

D. Albuterol

Question 29.16 Fifteen days into treatment she develops a severe headache. Her neurologic and fundoscopic evaluations are normal. Review of her morning laboratory tests reveal a WBC of 0.6/μL, hematocrit of 9.8, platelet count of 25,000/μL, INR of 1.4, and fibrinogen of 190. You obtain a noncontrast head computed tomography scan but are more worried that this is a result of:

A. Relapse.

B. ATRA.

C. Idarubicin.

D. Transfusion reaction.

Question 29.17 Six months later you are reviewing her chart. Her CBC has normalized, and she has tolerated consolidation therapy. Her most recent PCR showed no sign of residual disease. You are most concerned about relapse because of:

A. Her microgranular variant presentation.

B. Her presenting coagulopathy.

C. Her presenting WBC.

D. Her ethnicity.

Question 29.18 Three years later she presents to clinic with an elevated leukocyte count. PCR of peripheral blood confirms the presence of her initial t(15;17) translocation. Treatment options at this point include which of the following?

A. Arsenic trioxide

B. Gemtuzumab ozogamicin

C. Autologous transplant after achieving CR

D. All of the above

Question 29.19 A 30-year-old, otherwise healthy woman is diagnosed with AML. Cytogenetics reveal inv(16). She undergoes induction therapy. Day-14 bone marrow biopsy shows an ablated marrow. Her day-45 marrow shows restored cellularity without evidence of disease. Repeat cytogenetics do not reveal the inv(16) rearrangement. Appropriate consolidation therapy would be:

A. Allogeneic transplant if a matched sibling donor is available.

B. High-dose cytarabine (HIDAC) 3 g/m² every 12 hours on days 1, 3, and 5 for four 28-day cycles.

C. Intermediate-dose cytarabine (IDAC) 300 mg/m² every 12 hours on days 1, 3, and 5 for four 28-day cycles.

D. Arsenic 0.15 mg/kg on days 1 to 5 for four 28-day cycles.

Question 29.20 Which of the following regimens require graft-versus-tumor effects to treat residual AML blasts?

A. Busulfan-cyclophosphamide

B. Cyclophosphamide-TBI

C. Busulfan-fludarabine

D. Busulfan-VP16

Question 29.21 Central nervous system (CNS) prophylaxis should be considered in which of the following patients?

A. A 25-year-old Hispanic woman with APL who presents with a WBC of 2500/μL, an INR of 2.5, and fibrinogen of 100

B. A 78-year-old man with AML evolved from MDS

C. A 30-year-old woman with AML who develops headaches while receiving ondansetron for nausea on day 9 of induction therapy

D. A 20-year-old man with Down syndrome and ALL who presents with leukocytosis (WBC 120,000/μL)

Question 29.22 Dexamethasone has replaced prednisone in ALL induction therapy because of improved penetration in which tissue?

A. Testes

B. Spleen

C. Bone marrow

D. Brain

Question 29.23 How long should maintenance therapy for ALL with daily 6-mercaptopurine, weekly methotrexate, and monthly vincristine and prednisone be continued?

A. 6 months

B. 12 months

C. 24 to 36 months

D. Until relapse

Question 29.24 Which of the following targeted agents have been shown to be beneficial in adult ALL?

A. Imatinib

B. Alemtuzumab

C. Gemtuzumab ozogamicin

D. Sunitinib

Question 29.25 For which of the following patients would you consider myeloablative stem cell transplantation in CR1?

A. A 30-year-old woman with inv(16) AML-M4 with eosinophilia

B. A 50-year-old man with complex cytogenetics AML-M1

C. A 65-year-old man with complex cytogenetics AML-M1

D. A 50-year-old woman with t(15;17) AML-M3

Question 29.26 Which of the following statements is TRUE regarding the nucleophosmin 1 (NPM1) mutation in AML? (Select two correct responses)

A. In normal karyotype AML, NPM1 mutation without FLT3-ITD confers a prognosis similar to good-risk cytogenetics.

B. NPM1 mutations are found mostly in patients with AML with normal karyotype.

C. NPM1 mutation combined with FLT3-ITD is associated with good prognosis.

D. All the above

Stay updated, free articles. Join our Telegram channel

Join