Acute Lymphocytic Leukemia:
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You are here: Home » Medicine Notes , Pathology Notes » Acute Lymphocytic Leukemia:
Most common childhood malignancy, accounts for ~30% of all childhood cancers
Peak incidence ages 2-5 y/o
Improved treatment->5/yr survival has increased to 78-85%
Treatment overview:
1) Remission induction
-Goal: decrease blast cell percentage in bone marrow to 5% or lower
1 month scheduled vincristine, prednisone and L-asparaginase
2) Consolidation/CNS prophylaxis
-Combined antimetabolites /other agents with intrathecal chemotherapy
Ie cytarabine, etoposide (epipidophylotoxin), MTX, anthracyclines and alkylating agents (ie. cyclophosphamide, usually reserved for more aggressive leukemia, higher incidence of side effects)
-Craniospinal or cranial radiotherapy (24 Gy), once considered standard of care, was reduced or eliminated from many CNS preventive therapy protocols in the early 1980s due to association with significant toxicity.
3) Maintenance/ continuous treatment
2 yrs, Methotrexate and 6-mercaptopurine
Recommendations for the Primary Care Provider During ALL Patient’s Chemotherapy:
-Encourage patients to return to their oncologist for scheduled visits and follow-up studies, both during and after chemotherapy
-Communicate regularly with patient’s oncologist to find out what their treatment regimen is or was.
-There is no standard oncology follow-up frequency, but patients are typically followed monthly for the first year after therapy and then less frequently for the next 2-4 yrs. After 3-5 yrs, followed on annual basis.
Immunizations: only inactive immunizations during chemotherapy. Live-virus vaccines (MMR, OPV, varicella) are contraindicated. Give missed vaccinations after chemo
-Cancer patients have variable response to immunizations given while immunosuppressed. Children with ALL may fail to maintain protective Ab titers after completion of chemo. Check Ab titers 3-6 mos after chemo completion
After ALL Patient’s Chemotherapy: Examine for disease recurrence and screen for long-term side effects:
Late effects-any chronic or late occurring physical or psychosocial outcome persisting or developing more than 5 yrs after the diagnosis of the cancer
| Late Effect | Screening Recommendation |
| Cognitive dysfunction | Neurocognitive testing, before beginning school and q 3-4 yrs thereafter, more freq if abnL. |
| Psychosocial well-being | Yearly psychosocial assessment, with attn to educational and/or vocational progress, depression, anxiety, post-traumatic stress and social withdrawal |
| Obesity, physical inactivity and risk of premature Cardiovascular disease | Counseling on proper diet and exercise |
| Osteopenia and osteoporosis | -Counseling on Calcium intake, smoking avoidance/cessation and the importance of exercise in increasing bone density -Bone density eval (DEXA scan or CT) at baseline; repeat as clinically indicated |
| Late effect | Screening Recommendation |
| GH deficiency | -Dx by h/o cranial or total body radiation, slow growth rate, normal weight gain, no intercurrent illness, delayed bone maturation, IGF-1 and IGFBP-3 less than –1 SD for age, provocative GH testing. -GH tx can result in normalization of height; usually stopped at 18y/o |
| Anthracycline-induced cardiomyopathy | Echo q2-3 yrs in pt’s who received more than 300mg/m2 cumulative anthracycline |
| Infectious-in patients who received blood/serum products prior to appropriate screening | -anti-HCV in pts who received blood products before July 1972 -HIV 1&2 in pts who received tx btn 1977-1985 |
| ALL relapse (bone marrow, CNS) | -Hx: Fever, malaise, bleeding and bone pain. Labs: yearly CBC/differential |
| Second Malignant Neoplasms -therapy-related AML -brain tumors | Yearly, up to 10 yrs after exposure to agent: AML -Hx: fatigue, bleeding, easy bruising -PEX: dermatologic exam (pallor, petechiae, purpura) -Labs: CBC/differential Brain tumor: -Hx: Headaches, vomiting, cognitive, motor or sensory deficits, seizures -PEX: Neurologic examHISTORY- HISTORY |
| Fertility and Reproduction -Testicular/ovarian dysfunction, delayed/arrested puberty, infertility -Hypogonadism, oligospermia, azoospermia (male) -Premature menopause (female) | -Hx: Pubertal onset and tempo, sexual function, medication use impacting sexual function, menstrual/pregnancy hx (female), -PEX: Tanner stage, yearly until sexually mature -Labs: Baseline FSH, LH and Estradiol (female) at age 13 and as clinically indicated. -Baseline FSH, LH and Testosterone (male) at age 14 and as clinically indicated. -Semen analysis as indicated. -Counsel that there is no known increased risk of congenital abnLity in offspring of ALL survivors |
| Ocular abnormalities (cataracts, lens densities) | Yearly vision and cataract screening |
| Dental and Periodontal Disease -Abnormalities include tooth agenesis, arrested tooth development, microdontia and enamel dysplasia | Oral exam yearly, and dental cleaning every 6 mos |
| Thyroid Related Disorders (Hypothyroid) | Yearly TSH and T4 |
| Pulmonary late effects - Increased prevalence of mild, usually subclinical restrictive pulmonary disease | -Hx (yearly): cough, SOB, DOE, WheezingTORY -Pulm exam: yearly -Studies: CXR, PFTs, baseline and as clinically indicated |
| Liver dysfunction (non-hepatitis C) | Yearly LFTs |
| Urologic late effects -Hemorrhagic cystitis, bladder CA,dysfunctional voiding, hydronephrosis 2/2 Cyclophosphamide | -ROS should include voiding problems -Yearly UA |
| Dermatologic late effects Alopecia, nail dysplasia, vitiligo, scleroderma | Yearly dermatologic exam |
Sources:
Oettinger, KC et al. The Journal of Family Practice, Dec 2000; 49:1133. Providing Primary Care for Long-Term Survivors of Childhood Acute Lymphoblastic Leukemia
Children’s Oncology Group, www.survivorshipguidelines.org
Up to Date: Overview of the treatment and outcome of ALL in Children, www.utdol.com
Category: Medicine Notes , Pathology Notes
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