Philadelphia Chromosome–Negative B-Cell Acute Lymphoblastic Leukemia in Adolescents and Young Adults

Amy Y. Wang, MD, MPH; Lori S. Muffly, MD, MS; Wendy Stock, MD


J Oncol Pract. 2020;16(5):231-238. 

In This Article

Special AYA Topics

The care of the AYA patient would not be complete without considering the issues that arise from the life disruptions brought on by ALL at a time of critical physical and psychological development. Together, the direct and indirect effects of treatment contribute to substantial levels of psychological distress.

Education, Career, and Finances

Studies of AYA survivors have found a comparatively lower level of educational attainment,[50] higher unemployment rates,[51] and more issues with attention, memory, and health even after returning to work.[52] The financial toxicity of cancer treatment can be crippling. Approximately one third of survivors reported borrowing money or going into debt, 47% had borrowed more than $10,000, and 5% went bankrupt.[53] Many have also reported difficulty acquiring and maintaining health insurance,[54] which directly correlates with survival.[55]


The formation of social and romantic relationships is critical to one's psychological development and self-identity. However, disruptions to this process can lead to increased dependence on family members, feelings of isolation, loss of autonomy, and decreased self-esteem.[50,54,56,57] Additional changes in physical appearance, such as weight gain from steroids, hair loss from chemotherapy, and scars from procedures, are associated with decreased self-esteem, social isolation, and poor adherence.[56,58]

Fertility Preservation

Fertility preservation should be discussed with every AYA patient, because cancer treatments can cause temporary or permanent gonadal failure. However, one study found that the rates of provider discussions varied widely from 17% to 82% depending on tumor type and sex.[59] Patients have cited cost, insurance-related issues, and not wanting to delay start of treatment as reasons for not pursuing fertility preservation.[59] Sperm banking costs from $1,000 to $1,500, with annual storage costs of $200 to $400; in contrast, one cycle of embryo preservation costs on average $12,700.[57,60] Only Connecticut and Rhode Island have passed legislation mandating insurance coverage for fertility preservation; elsewhere, insurance coverage is rare and remains an issue of national debate.[60]

Late-onset Health Effects

The topic of late effects in AYA cancer survivors was recently comprehensively reviewed by Baker and Syrjala.[53] Notable treatment-related long-term complications include anthracycline-associated cardiotoxicity, vincristine-induced neuropathy, premature cardiovascular disease, metabolic syndrome, obesity, gonadal dysfunction, osteonecrosis, asthma, and secondary malignancies.[51,53] AYA survivors also have higher rates of tobacco use, disability, mental health issues, and access to care issues,[51] highlighting the importance of long-term follow-up and survivorship care.

Psychological Issues

AYAs have a high level of unmet psychosocial and supportive care needs.[57,61,62] Symptoms of depression, anxiety, and post-traumatic stress were prevalent in one third of AYAs according to one study.[63] Not only was there a delay in reporting psychological distress by patients but providers also had a poor ability to recognize signs of distress.[63] In addition, there is a need to assess for neurocognitive changes that may occur as a result of the intensive chemotherapy plus CNS-directed treatment. Hence, the complexities of caring for AYAs with cancer necessitate a multidisciplinary approach, with a team of oncologists, oncology nurses, dieticians, physical therapists, psychologists, social workers, palliative care team, and fertility experts, preferably in a specialized AYA cancer center. AYA-specific training is becoming more prevalent to providers of all levels in the form of workshops, continuing education programs, fellowships, and support tools.[57]