Pediatric Acute Lymphoblastic Leukemia Exam Questions with Verified Answers (Graded A+)

Pediatric Acute Lymphoblastic Leukemia Exam Questions with Verified Answers (Graded A+) What is risk stratum of pediatric ALL using patient case? - Answers What labs are suggestive of ALL? - Answers WBC: 70.3 (5-15 is normal) Hgb: 5.8 (10.5-13.5) Plt: 36 (150 - 400) Blasts: 89.0 What is the etiology? - Answers most common pediatric malignancy (leading cause of childhood cancer death) overall survival rates currently ~90% peak incidence 2-5 years old boys girls, white hispanic Black, Asian higher incidence in industrial countries Diagnosis of ALL? - Answers symptoms are are non-specific procedures (bone marrow biopsy and lumbar puncture) Pathophysiology of ALL? - Answers T lymphocyte (10%) B lymphocyte (90%) What is CNS Status? - Answers CNS 1: no leukemia cells present on CSF studies CNS 2: less than 5 WBCs + Blasts on CSF studies CNS 3: greater than equal to 5 WBCs + Blasts on CSF studies What is important to know about intrathecal chemo? - Answers direct injection into CNS used for both treatment of CNS disease and prophylaxis against development of CNS disease (systemic chemo has limited CNS penetration) limited systemic penetration *most common - intrathecal cytarabine* - may also give methotrexate or "triple IT" (cytarabine + methotrexate + hydrocortisone) What is the treatment of TLS? - Answers start hydration at 1.5 to 2x maintenance rate give *single dose of raburicase 0.2mg/kg; cap dose at 6mg* --expensive / will not get rid of new uric acid production *start allopurinol 10/mg/kg/day divided TID* --inhibit xanthine oxidase and prevent uric acid crystals *start sevelamer 800mg TID with meals* -- prevent calcium / phosphorus precipitate formation -- does not do anything for the phos that is there (this is a phos binder) What is TLS? - Answers tumor lysis syndrome (fairly common in ALL) tumor cells lyse (either spontaneously or as a result of chemotherapy) -- will release all of the intracellular components (DNA) high K Low Ca High phos High uric acid (uric acid crystals) *protect kidneys, protect the heart (because high K can cause arrhythmia / death) What are the ALL risk factors? - Answers Standard Risk: -ALL of the following: 1 to 10 years old, WBC less than 50k, CNS-1 or 2, B-ALL HIGH RISK -ANY of the following: less than 1 OR 10 years or older, WBC greater than or equal to 50k, CNS-3, T-ALL What is important to know about B-ALL cytogenetics? - Answers B-ALL genetic mutations exists (some are more or less favorable) What are the three phases of ALL therapy and the goals? - Answers 1. Induction: induce remission (wipe out active malignant cells) 2. Consolidation: eliminate residual disease, prevent CNS disease 3. Maintenance: ensure durable remission What is important to know about induction chemotherapy regimen? - Answers Traditional 4-drug induction consists of: 1. Steroid. *prednisone vs dexamethasone (used as anti-emetic* -induce apoptosis of leukemia cells (potentially through inhibition of glucose transport or phosphorylation) -ADEs: agitation / insomnia, hyperglycemia, hypertension, hyperphagia 2. Asparaginase *pegaspargase or calaspargase 3. *Vincristine* 4. Anthracycline *doxorubicin or daunorubicin* What are important disguising points about prednisone vs dexamethasone? - Answers Prednisone: - given for patients older than 10 - slightly less efficacious