AYA Patient Case in ALL

How I Used Pegylated Asparaginase to Treat a Young Adult With ALL

Released: November 07, 2022

Expiration: November 06, 2023

Daniel J. DeAngelo, MD, PhD

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Key Takeaways

Pediatric or pediatric-inspired regimens containing asparaginase are effective treatments for adolescent/young adult and some adult patients with acute lymphoblastic leukemia.
Despite being complicated regimens that are challenging to administer, completion of asparaginase-based therapy is possible and associated with good outcomes.
To learn how experts mitigate challenges associated with delivery of asparaginase-containing regimens to adolescent/young adult and adult patients with acute lymphoblastic leukemia, register now for our symposium at the 2022 American Society of Hematology Annual Meeting and Exposition.

Pediatric-inspired chemotherapy regimens containing asparaginase are an established standard of care for the treatment of acute lymphoblastic leukemia (ALL) in adolescent/young adult (AYA) and some adult patients, but they are complicated regimens that are challenging to administer. In this commentary, I describe a best-case scenario example from my clinic of an AYA patient with newly diagnosed ALL who was successfully treated with pegylated asparaginase.

Case Presentation: AYA Patient With Newly Diagnosed ALL
A young woman presented to my clinic with a 2-week history of fatigue and dizziness that she initially attributed to studying for midterm exams. At the time, she was a 21-year-old junior in college who was visiting her parents in the Boston area during spring break. She also presented with gum bleeding and wet purpura in her mouth after a dental procedure, for which the dentist advised her to go to urgent care. At urgent care, she was noted to have an elevated white blood cell count of 85,000/mm³, and the differential showed that these were primarily in blast form (92%). Her hemoglobin was 10 g/dL, and her platelet count was 17,000/mm³.

Workup
The patient was referred to the Dana-Farber Cancer Institute, where a bone marrow examination confirmed a population of >90% immature blast cells. On immunophenotypic analysis, the blasts were dimly positive for CD45, strongly positive for CD10, CD19, HLA-DR, and TdT, and negative for CD20 myeloid antigens, and surface immunoglobulin. Blood PCR testing was negative for BCR-ABL1, and cytogenetic analysis showed no Philadelphia chromosome but did reveal der19 t(1;19). A gene fusion assay confirmed the translocation, which involved an E2A-PBX1 gene fusion. She was diagnosed with Philadelphia-negative B-cell ALL.

Treatment
With this patient being 21 years of age, she could have been seen in either the adult or pediatric clinic. At our institution, we commonly treat patients over 18 years of age in the adult clinic. Based on data from retrospective analyses and many prospective clinical trials demonstrating a survival benefit with a pediatric regimen or pediatric-inspired regimen in adult patients younger than 40 years of age, I recommended that she be treated with an intensive chemotherapy course including pegylated asparaginase.

The patient was enrolled on our Dana-Farber pediatric trial and achieved a morphologic remission with minimal residual disease negativity by multicolor flow cytometry after induction chemotherapy. We continued the pegylated asparaginase as consolidation with central nervous system prophylaxis, then proceeded with prolonged maintenance therapy for 2 years. We did not recommend a stem cell transplant for this patient.

Follow-up
She completed all doses of pegylated asparaginase without delays or complications and remains remarkably well today. She is now 12 years out from her initial diagnosis and recently got married.

This patient case provides a best-case scenario example of a favorable outcome with the use of a pediatric-inspired regimen containing asparaginase in an AYA patient with Philadelphia chromosome–negative ALL. However, we know that use of asparaginase in clinical practice is not without its challenges.

To Learn More, Attend Our Symposium at ASH 2022!
Want to learn how experts mitigate challenges associated with delivery of pediatric-inspired chemotherapy regimens containing asparaginase to AYA and adult patients with ALL? Join me and my colleagues Kjeld Schmiegelow, MD, DMSci, and Emily Curran, MD, in New Orleans or online on Friday, December 9, for a CME-certified symposium titled, “Acute Lymphoblastic Leukemia: Data and Case Discussion on the Use of Asparaginase Therapy in Young and Older Adults” at the American Society of Hematology Annual Meeting and Exposition.

During the symposium, we will review the latest data and discuss patient cases, including:

Why we should be using asparaginase-based regimens
Guidance on strategies to increase completion of asparaginase
Is it hypersensitivity or intolerance or inactivation?
Prevention and management of hypersensitivity reactions
Risk factors for asparaginase toxicities
Management of asparaginase-related toxicities such as hepatic toxicity and thrombosis
When to adjust the asparaginase dose and when to switch asparaginase formulations
What is the age cutoff for use of pediatric-inspired regimens with asparaginase?

Click here to register!

Your Thoughts?
What challenges have you encountered when using asparaginase-based regimens to treat your AYA and adult patients with newly diagnosed ALL? Leave a comment in the discussion box below!

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Poll

1.

Are you using pediatric-inspired asparaginase-based regimens in clinical practice for your AYA and adult patients with newly diagnosed ALL?

A. Yes
B. Yes, but only in patients younger than 40 years of age
C. Yes, but only in patients younger than 30 years of age
D. No
E. Other

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