Jun 03, 2023
ASCO seeks to advance the education of all oncology professionals and ultimately facilitate and support enhanced patient care. The ASCO Oncology Self-Assessment Series on ASCO Connection consists of free case-based multiple-choice practice questions, educational links, and answer rationales from ASCO-SEP.
Learn more about ASCO’s Educational products, such as the 2022 ASCO-SEP Digital Subscription, which includes the digital book, access to education courses and virtual meeting-related content, plus over 1,000 practice questions in the Question Bank. Oncology trainees and training program directors can visit Education Essentials for Oncology Fellows (EEOF) to learn more and register for the 2022-2023 cycle.
Correct answers are listed at the bottom of the page.
Question 1: Leukemia
A 73-year-old woman presents to the emergency department with a fever, mild confusion, and dehydration. Laboratory studies show a white blood count of 38,000 cells/μL, platelet count of 33,000 cells/μL, and hemoglobin level of 6.8 g/dL. A blood smear reveals abnormal promyelocytes with numerous red to purple cytoplasmic granules with dense Auer rods. There are some scattered questionable schistocytes. Physical examination reveals extensive bruising on her arms, thighs, and trunk areas. Fluorescence in situ hybridization (FISH) shows the presence of PML-RARA translocation.
Which of the following is the most appropriate next step?
- Start induction chemotherapy with daunorubicin and cytarabine
- Administer eculizumab
- Start oral all-trans-retinoic acid (ATRA) and correct coagulopathy
- Place a Shiley catheter and start plasma exchange
Question 2: Lymphoma
A 23-year-old man presents 42 days post–double umbilical cord transplant for relapsed acute myeloid leukemia (AML) in second remission. Primary graft-versus-host disease (GVHD) prophylaxis included cyclosporine, antithymocyte globulin (ATG), and mini-methotrexate. He has no evidence of GVHD. Laboratory testing shows a leukocyte count of 2,300 cells/µL, hemoglobin level of 8.9 g/dL, and platelet count of 45,000 cells/µL. A CT scan of the neck is performed for dysphagia and reveals multiple enlarged cervical lymph nodes. An excisional biopsy shows Epstein-Barr virus (EBV)–positive plasmablasts consistent with polymorphic posttransplant lymphoproliferative disorder (PTLD). Current medications include cyclosporine, acyclovir, posaconazole, and ursodiol.
Which of the following is the most appropriate next step?
- Stop acyclovir and start ganciclovir
- Rituximab
- Stop cyclosporine
- External-beam radiation therapy to cervical lymph nodes
Question 1 Rationale and References
Correct answer: C. Start oral all-trans-retinoic acid (ATRA) and correct coagulopathy
Rationale: Owing to potentially rapid fatal coagulopathy, treatment must begin before diagnosis is confirmed in patients with suspected acute promyelocytic leukemia (APL). The treatment of patients with APL represents a true emergency primarily because of bleeding, which continues to represent a major cause of early death. Once APL is suspected based on clinical findings and the peripheral blood smear (even without waiting for a bone marrow examination), and before cytogenetic or molecular studies confirm the diagnosis, ATRA should be started at the standard dose of 45 mg/m2 per day in divided doses and given emergently, both to resolve the coagulopathy as well as to initiate induction therapy. Total white blood cell count of over 10,000 cells/μL places the patient in the high-risk category. In this setting, an anthracycline may be added to ATRA and arsenic trioxide; however, cytarabine is not typically included.
References
- Osman AEG, Anderson J, Churpek JE, et al. Treatment of acute promyelocytic leukemia in adults. J Oncol Pract. 2018 Nov;14(11):649-57. DOI: https://doi.org/10.1200/JOP.18.00328
- Lo-Coco F, Avvisati G, Vignetti M, et al. Retinoic acid and arsenic trioxide for acute promyelocytic leukemia. N Engl J Med. 2013 Jul 11;369(2):111-21. DOI: https://doi.org/10.1056/NEJMoa1300874
Question 2 Rationale and References
Correct answer: C. Stop cyclosporine
Rationale: PTLD is an uncommon complication after solid organ and hematopoietic stem cell transplant. Risk factors include prolonged immunosuppression, use of T-cell depleted grafts, umbilical cord and haploidentical transplants, and EBV seropositivity before transplant. There are several subtypes to have early lesions, polymorphic PTLD, and monomorphic PTLD. For EBV-positive PTLD, the preferred treatment is reduction or discontinuation of immune suppression. This results in regression of PTLD in 23% to 50% of cases. Little data support the use of antiviral agents to treat PTLD, and this patient’s cytopenia limits the usage of ganciclovir. Rituximab is effective for pre-emptive therapy when the viral load of EBV is increasing and can be used as treatment if the withdrawal of immune suppression is ineffective. Radiation therapy can be considered for central nervous system involvement but has no defined role in this patient’s presentation.
References
- Petrara MR, Giunco S, Serraino D, Dolcetti R, De Rossi A. Post-transplant lymphoproliferative disorders: from epidemiology to pathogenesis-driven treatment. Cancer Lett. 2015;369(1):37-44. DOI: https://doi.org/10.1016/j.canlet.2015.08.007
- Dierickx D, Habermann TM. Post-transplantation lymphoproliferative disorders in adults. N Engl J Med. 2018;378(6):549-62. DOI: https://doi.org10.1056/NEJMra1702693