AOCNP Hematologic Malignancies 5 — Questions and Answers
Question 1: A 60-year-old with newly diagnosed AML has FLT3-ITD mutation. Which agent should be added to standard induction chemotherapy based on current NCCG guidelines?
- Venetoclax
- Midostaurin (Correct answer)
- Enasidenib
- Glasdegib
Correct answer: Midostaurin
Midostaurin, a FLT3 inhibitor, significantly improves OS when added to standard 7+3 induction in FLT3-mutated AML (RATIFY trial).
Question 2: A patient with Hodgkin lymphoma relapses after ABVD chemotherapy. PET-CT confirms relapsed disease. What is the standard treatment approach for transplant-eligible patients?
- Brentuximab vedotin monotherapy
- Salvage chemotherapy followed by autologous stem cell transplant (Correct answer)
- Allogeneic stem cell transplant upfront
- Palliative radiation only
Correct answer: Salvage chemotherapy followed by autologous stem cell transplant
Relapsed/refractory Hodgkin lymphoma in transplant-eligible patients is treated with platinum-based salvage chemotherapy followed by high-dose chemotherapy and autologous SCT.
Question 3: Which clinical feature distinguishes essential thrombocythemia (ET) from a reactive thrombocytosis?
- Platelet count >600,000/μL
- Presence of JAK2, CALR, or MPL mutation (Correct answer)
- Splenomegaly on exam
- Elevated C-reactive protein
Correct answer: Presence of JAK2, CALR, or MPL mutation
Driver mutations (JAK2 V617F, CALR, MPL) confirm clonal thrombocytosis in ET; reactive thrombocytosis has no clonal mutation.
Question 4: A patient with Waldenström macroglobulinemia presents with episodic Raynaud phenomenon, urticaria, and hyperviscosity. Which immunoglobulin class is responsible?
- IgA
- IgG
- IgM (Correct answer)
- IgE
Correct answer: IgM
Waldenström macroglobulinemia is characterized by a monoclonal IgM paraprotein; its large pentameric structure causes hyperviscosity and cold agglutinin/cryoglobulin phenomena.
Question 5: A patient with T-cell acute lymphoblastic leukemia (T-ALL) has a mediastinal mass causing superior vena cava syndrome. Which chemotherapy backbone is most appropriate for induction?
- CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone)
- Hyper-CVAD or BFM-based multi-agent protocol (Correct answer)
- Fludarabine-based regimen
- Carfilzomib plus dexamethasone
Correct answer: Hyper-CVAD or BFM-based multi-agent protocol
T-ALL is treated with intensive multi-agent protocols such as Hyper-CVAD or BFM, which incorporate L-asparaginase and CNS prophylaxis.
Question 6: In myelodysplastic syndrome (MDS), which cytogenetic finding is associated with the best prognosis and typically responds to lenalidomide?
- Monosomy 7
- Del(5q) (Correct answer)
- Complex karyotype (≥3 abnormalities)
- Del(17p)
Correct answer: Del(5q)
Isolated del(5q) MDS has an excellent prognosis and is uniquely sensitive to lenalidomide, which targets the haploinsufficient CSNK1A1 gene on 5q.
Question 7: A 35-year-old with Burkitt lymphoma receives CODOX-M/IVAC chemotherapy. Which prophylactic medication is most critical to prevent a life-threatening metabolic complication?
- Acyclovir
- Rasburicase (Correct answer)
- Fluconazole
- Trimethoprim-sulfamethoxazole
Correct answer: Rasburicase
Burkitt lymphoma has an extremely high proliferative rate; rasburicase rapidly degrades uric acid to prevent severe tumor lysis syndrome during induction.
AOCNP Hematologic Malignancies 5
A 60-year-old with newly diagnosed AML has FLT3-ITD mutation.
Which agent should be added to standard induction chemotherapy based on current NCCG guidelines?