AOCNP Hematologic Malignancies 4 — Questions and Answers
Question 1: A patient with CLL is found to have del(17p) on FISH testing. Which targeted therapy is the preferred first-line agent for this high-risk mutation?
- Ibrutinib (Correct answer)
- Fludarabine/cyclophosphamide/rituximab (FCR)
- Chlorambucil
- Bendamustine plus rituximab
Correct answer: Ibrutinib
Del(17p) CLL is resistant to chemoimmunotherapy due to loss of p53; ibrutinib (a BTK inhibitor) is preferred first-line.
Question 2: Which of the following is the most common cytogenetic abnormality in multiple myeloma that confers a standard-risk prognosis?
- t(4;14)
- del(17p)
- Hyperdiploidy (Correct answer)
- t(14;16)
Correct answer: Hyperdiploidy
Hyperdiploidy, typically involving odd-numbered chromosomes, is the most common cytogenetic finding in myeloma and is associated with standard risk.
Question 3: A patient on venetoclax for AML develops tumor lysis syndrome (TLS). Which lab finding is NOT a feature of TLS?
- Hyperkalemia
- Hyperphosphatemia
- Hypercalcemia (Correct answer)
- Hyperuricemia
Correct answer: Hypercalcemia
TLS causes hypocalcemia (not hypercalcemia) because phosphate released from lysed cells binds calcium, lowering serum levels.
Question 4: A 45-year-old with relapsed/refractory diffuse large B-cell lymphoma (DLBCL) achieves complete remission after CAR-T cell therapy. What is the most serious early toxicity to monitor for in the first 2 weeks?
- Hemolytic uremic syndrome
- Cytokine release syndrome (CRS) (Correct answer)
- Graft-versus-host disease
- Progressive multifocal leukoencephalopathy
Correct answer: Cytokine release syndrome (CRS)
CRS is the most common and potentially life-threatening early toxicity after CAR-T therapy, caused by massive cytokine release from activated T cells.
Question 5: In the revised International Prognostic Index (R-IPI) for DLBCL, which of the following is NOT one of the five adverse prognostic factors?
- Age >60 years
- Elevated LDH
- Bone marrow involvement (Correct answer)
- ECOG performance status >1
Correct answer: Bone marrow involvement
The R-IPI uses age >60, elevated LDH, ECOG PS >1, Ann Arbor stage III/IV, and >1 extranodal site; bone marrow involvement per se is not a standalone factor.
Question 6: A patient with polycythemia vera (PV) develops a new stroke. Which mutation drives the majority of PV cases and is used for diagnosis?
- BCR-ABL1
- JAK2 V617F (Correct answer)
- CALR exon 9
- MPL W515L
Correct answer: JAK2 V617F
JAK2 V617F is present in >95% of PV cases and is central to the 2016 WHO diagnostic criteria.
Question 7: Which surface marker combination is most characteristic of hairy cell leukemia (HCL)?
- CD5+, CD23+, CD19+
- CD19+, CD20+, CD11c+, CD25+, CD103+ (Correct answer)
- CD3+, CD4+, CD30+
- CD34+, CD117+, CD13+
Correct answer: CD19+, CD20+, CD11c+, CD25+, CD103+
HCL classically expresses CD19, CD20, CD11c, CD25, CD103, and annexin A1, distinguishing it from other B-cell lymphoproliferative disorders.
AOCNP Hematologic Malignancies 4
A patient with CLL is found to have del(17p) on FISH testing.
Which targeted therapy is the preferred first-line agent for this high-risk mutation?