AOCNP Hematologic Malignancies 1 — Questions and Answers
Question 1: Which type of leukemia is the most common leukemia in adults in the United States?
- Acute lymphoblastic leukemia (ALL)
- Chronic lymphocytic leukemia (CLL) (Correct answer)
- Acute myeloid leukemia (AML)
- Chronic myeloid leukemia (CML)
Correct answer: Chronic lymphocytic leukemia (CLL)
Chronic lymphocytic leukemia (CLL) is the most common adult leukemia in Western countries, accounting for approximately 25–30% of all leukemias.
Question 2: The Philadelphia chromosome, resulting from a t(9;22) translocation that produces the BCR-ABL fusion gene, is the hallmark of which leukemia?
- Acute myeloid leukemia (AML)
- Chronic lymphocytic leukemia (CLL)
- Chronic myeloid leukemia (CML) (Correct answer)
- Hairy cell leukemia
Correct answer: Chronic myeloid leukemia (CML)
The Philadelphia chromosome [t(9;22)] producing BCR-ABL is the defining molecular abnormality of CML and the target of tyrosine kinase inhibitors like imatinib.
Question 3: A patient is diagnosed with acute promyelocytic leukemia (APL, AML-M3). Which agent is a cornerstone of induction therapy that induces differentiation of malignant promyelocytes?
- Cytarabine (ara-C)
- Daunorubicin
- All-trans retinoic acid (ATRA) (Correct answer)
- Hydroxyurea
Correct answer: All-trans retinoic acid (ATRA)
ATRA targets the PML-RARA fusion protein in APL, promoting differentiation of malignant promyelocytes and is combined with arsenic trioxide or anthracyclines for induction.
Question 4: A patient with newly diagnosed AML has a FLT3-ITD mutation. What is the clinical significance of this finding?
- It confers a favorable prognosis and no additional targeted therapy is needed
- It is associated with poor prognosis and warrants addition of a FLT3 inhibitor such as midostaurin (Correct answer)
- It indicates the patient has APL and should receive ATRA
- It predicts complete remission with standard chemotherapy alone
Correct answer: It is associated with poor prognosis and warrants addition of a FLT3 inhibitor such as midostaurin
FLT3-ITD mutations in AML are associated with high relapse rates and poor overall survival; adding midostaurin (a FLT3 inhibitor) to standard induction chemotherapy improves outcomes.
Question 5: Which of the following is the most accurate description of a complete cytogenetic response (CCyR) to imatinib therapy in CML?
- BCR-ABL transcript undetectable by PCR
- No Philadelphia chromosome-positive cells detected in cytogenetic analysis (0% Ph+ metaphases) (Correct answer)
- White blood cell count normalized to within normal limits
- Fewer than 35% Philadelphia chromosome-positive metaphases
Correct answer: No Philadelphia chromosome-positive cells detected in cytogenetic analysis (0% Ph+ metaphases)
Complete cytogenetic response (CCyR) is defined as 0% Philadelphia chromosome-positive metaphases on conventional cytogenetic analysis of bone marrow.
Question 6: The Rai and Binet staging systems are used to classify the prognosis of which hematologic malignancy?
- Chronic myeloid leukemia (CML)
- Hodgkin lymphoma
- Chronic lymphocytic leukemia (CLL) (Correct answer)
- Multiple myeloma
Correct answer: Chronic lymphocytic leukemia (CLL)
The Rai (US) and Binet (European) staging systems stratify CLL patients into risk groups based on lymphocytosis, lymphadenopathy, organomegaly, and cytopenias.
Question 7: Tumor lysis syndrome (TLS) is most likely to occur following the initiation of treatment in which clinical scenario?
- A patient with low-grade follicular lymphoma starting rituximab maintenance
- A patient with high-grade Burkitt lymphoma beginning chemotherapy (Correct answer)
- A patient with CML beginning imatinib in chronic phase
- A patient with multiple myeloma starting lenalidomide
Correct answer: A patient with high-grade Burkitt lymphoma beginning chemotherapy
Burkitt lymphoma has an extremely high proliferative rate; rapid cell lysis upon chemotherapy releases intracellular contents, causing the classic TLS electrolyte abnormalities (hyperkalemia, hyperphosphatemia, hypocalcemia, hyperuricemia).
AOCNP Hematologic Malignancies 1
Which type of leukemia is the most common leukemia in adults in the United States?