ITE Hematology and Oncology 5 — Questions and Answers
Question 1: A 72-year-old man with CLL has a rising lymphocyte count and new lymphadenopathy. Biopsy shows transformation to DLBCL with loss of CD5 expression and elevated LDH. What is this transformation called?
- Richter transformation (Correct answer)
- Accelerated phase CLL
- Prolymphocytic transformation
- Blastic transformation
Correct answer: Richter transformation
Richter transformation refers to the development of aggressive lymphoma (most commonly DLBCL) in a patient with CLL, carrying a poor prognosis.
Question 2: A patient with AML achieves complete remission after induction chemotherapy. Cytogenetics show inv(16). Which post-remission strategy is preferred?
- Allogeneic stem cell transplant
- High-dose cytarabine consolidation (Correct answer)
- Autologous stem cell transplant
- Maintenance azacitidine
Correct answer: High-dose cytarabine consolidation
Inv(16) is a favorable-risk cytogenetic abnormality in AML; high-dose cytarabine consolidation achieves excellent outcomes without the added toxicity of transplant.
Question 3: A 50-year-old woman presents with venous thrombosis at an unusual site (portal vein). Testing reveals the JAK2 V617F mutation. What underlying condition is most likely?
- Essential thrombocythemia
- Myeloproliferative neoplasm (polycythemia vera) (Correct answer)
- Factor V Leiden deficiency
- Antiphospholipid syndrome
Correct answer: Myeloproliferative neoplasm (polycythemia vera)
JAK2 V617F is found in ~95% of polycythemia vera cases and is the most common MPN associated with splanchnic vein thrombosis.
Question 4: A 25-year-old man presents with bilateral testicular germ cell tumor markers showing elevated AFP and beta-hCG. Staging CT shows retroperitoneal lymph nodes up to 3 cm but no visceral metastases. What is the first-line treatment?
- Orchiectomy followed by radiation alone
- Orchiectomy followed by BEP chemotherapy (Correct answer)
- BEP chemotherapy without surgery
- Orchiectomy and retroperitoneal lymph node dissection only
Correct answer: Orchiectomy followed by BEP chemotherapy
For intermediate or high-risk nonseminomatous GCT with elevated markers and lymph node involvement, orchiectomy followed by BEP (bleomycin, etoposide, cisplatin) is standard.
Question 5: A patient receiving cisplatin-based chemotherapy develops a serum creatinine rise from 0.9 to 2.1 mg/dL. Urinalysis shows granular casts and low urine sodium. What is the mechanism of nephrotoxicity?
- Prerenal azotemia from vomiting
- Direct proximal tubular cell injury (Correct answer)
- Interstitial nephritis from hypersensitivity
- Obstructive uropathy from uric acid crystals
Correct answer: Direct proximal tubular cell injury
Cisplatin causes direct proximal tubular injury through accumulation of platinum-DNA adducts, leading to tubular necrosis.
Question 6: A 40-year-old woman with myelodysplastic syndrome has a del(5q) as the sole cytogenetic abnormality, transfusion-dependent anemia, and platelet count of 450,000/μL. Which agent is specifically indicated?
- Azacitidine
- Lenalidomide (Correct answer)
- Decitabine
- Erythropoiesis-stimulating agents
Correct answer: Lenalidomide
Lenalidomide is specifically approved for transfusion-dependent MDS with isolated del(5q) and produces high rates of transfusion independence.
Question 7: A 55-year-old man with metastatic renal cell carcinoma (clear cell histology) has good performance status and no prior therapy. Which combination is preferred first-line based on current evidence?
- Sunitinib monotherapy
- Nivolumab plus ipilimumab or pembrolizumab plus axitinib (Correct answer)
- Temsirolimus monotherapy
- Bevacizumab plus interferon-alpha
Correct answer: Nivolumab plus ipilimumab or pembrolizumab plus axitinib
Checkpoint inhibitor-based combinations (e.g., nivolumab/ipilimumab or pembrolizumab/axitinib) have demonstrated superior overall survival compared to sunitinib in first-line clear-cell RCC.
ITE Hematology and Oncology 5
A 72-year-old man with CLL has a rising lymphocyte count and new lymphadenopathy.
Biopsy shows transformation to DLBCL with loss of CD5 expression and elevated LDH.
What is this transformation called?