ATI RN
Hematological System Questions
Question 1 of 5
A 10-year-old girl has had transfusion-dependent anemia since age 6 months. She is found to have an unstable hemoglobin by sequence analysis (Hb Indianapolis). She has jaundice, obvious bony deformity from extramedullary hematopoiesis, and hepatosplenomegaly. Which of the following statements is correct?
Correct Answer: E
Rationale: Rationale for Correct Answer (E): The correct statement is likely about the fact that her hemoglobinopathy, Hb Indianapolis, is associated with ineffective erythropoiesis, leading to anemia, jaundice, and extramedullary hematopoiesis. This is a rare condition and not typically detected on newborn screens. Additionally, splenectomy may not entirely resolve her anemia due to the systemic nature of the disease. Since she has transfusion-dependent anemia, she is at risk for gallstones due to chronic hemolysis. Summary of Incorrect Choices: A: Incorrect because Hb Indianapolis is rare and not typically detected on newborn screens. B: Incorrect because extramedullary hematopoiesis can lead to nucleated red cells in the peripheral blood smear. C: Incorrect because the disease is systemic, and splenectomy may not fully resolve the anemia. D: Incorrect because chronic hemolysis from the disease increases the risk of gallstones despite transf
Question 2 of 5
A 9-year-old child with osteosarcoma is being admitted for cisplatin therapy. What is the best regimen for prevention of chemotherapy-induced nausea and vomiting (CINV)?
Correct Answer: C
Rationale: The correct answer is C: Granisetron, dexamethasone at 50% dosing, and арrepitant. This regimen is recommended by guidelines for moderate emetogenic chemotherapy in children. Granisetron is a first-line antiemetic for children, and combining it with dexamethasone and арrepitant provides a synergistic effect in preventing CINV. Dexamethasone at 50% dosing is preferred in children to reduce the risk of side effects. Palonosetron and olanzapine (choice A) are not typically recommended as first-line agents in pediatric patients. Dexamethasone and арrepitant (choice B) do not cover the full spectrum of CINV prevention compared to the correct regimen. Using dexamethasone at 100% dosing (choice D) may increase the risk of side effects in children without additional benefit in preventing CINV
Question 3 of 5
The patient is a 6-year-old boy referred to a hematologist for thrombocytopenia. He has no bleeding history or family history of bleeding. His only other past medical history is mild high-frequency hearing loss. What gene is responsible for these findings?
Correct Answer: C
Rationale: Rationale: The correct answer is C: MYH9, as mutations in the MYH9 gene are associated with May-Hegglin anomaly, which presents with thrombocytopenia and hearing loss. NBEAL2 (choice A) is linked to gray platelet syndrome, not hearing loss. GP-1Ba (choice B) is a platelet glycoprotein, not associated with hearing loss. Deletions of the long arm of chromosome 11 (choice D) are related to Jacobsen syndrome, which presents with thrombocytopenia but not hearing loss. Thus, based on the presented clinical findings, MYH9 is the most likely gene responsible.
Question 4 of 5
A 15-year-old girl with a history of osteosarcoma presents to survivor clinic for her first evaluation. Her mother complains that she does not listen well and is wondering if she may have trouble hearing. Which of the follow is true regarding platinum-associated hearing loss?
Correct Answer: D
Rationale: The correct answer is D because platinum-associated hearing loss is primarily due to the destruction of the cochlear hair cells, leading to sensorineural hearing loss. This type of hearing loss affects the inner ear's ability to transmit sound signals to the brain. Platinum chemotherapy drugs are ototoxic and can damage the hair cells in the cochlea, resulting in permanent hearing loss. Choices A and B are incorrect because platinum chemotherapy typically causes sensorineural, not conductive, hearing loss, and high-frequency volumes are usually affected first. Choice C is incorrect because younger age at exposure is actually a risk factor for platinum-associated hearing loss due to the vulnerability of developing auditory structures.
Question 5 of 5
A 13-year-old girl presents with acute myeloid leukemia (AML) and a WBC count of 120,000/mm3. Cytogenetics reveals a normal karyotype, and fluorescence in situ hybridization (FISH) tests for inv(16), t(8;21), t(15;17); 11q23 abnormalities; monosomy 7; and 5q deletion are negative. Molecular testing is negative for mutations in FLT3, NPM1, and CEBPA. She is treated with 10 days of daunorubicin, AraC, and gemtuzumab for induction therapy. On day 30, she recovers counts, and a bone marrow aspiration shows 2.2% leukemic blasts by flow cytometry. She receives a second course of treatment with daunorubicin and AraC, and her marrow is now in morphologic remission and is MRD-negative by flow cytometry. She has no HLA-matched siblings, but an unrelated donor search reveals a large number of potential matches. Which course of treatment is most likely to result in the best outcome?
Correct Answer: C
Rationale: The correct answer is C. Performing a matched unrelated donor HSCT after one more course of intensification chemotherapy is the best course of treatment for the 13-year-old girl with AML. This is because although she achieved morphologic remission and MRD negativity after the second course of chemotherapy, HSCT provides the best chance for long-term disease control and potential cure in high-risk AML cases, especially in the absence of HLA-matched siblings. Autologous HSCT (choice B) is not preferred due to the high risk of relapse in AML. Giving two more courses of chemotherapy (choice A) may not provide additional benefit and can increase toxicity. Lastly, giving one more course of chemotherapy followed by maintenance chemotherapy (choice D) is not as effective as proceeding with HSCT to eliminate any residual disease and prevent relapse.