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: Step 1: Hb Indianapolis is a rare unstable hemoglobin variant causing severe hemolytic anemia. Step 2: The clinical presentation includes jaundice, bony deformity, hepatosplenomegaly due to extramedullary hematopoiesis. Step 3: None of the choices directly address the specific characteristics of Hb Indianapolis. Step 4: Therefore, the correct answer must provide insight into the unique features of this condition. Step 5: Choice E is correct as it highlights that the clinical presentation of Hb Indianapolis is distinct from other beta-hemoglobinopathies. Step 6: Summary: Choices A, B, C, and D are incorrect as they do not address the specific features of Hb Indianapolis, unlike choice E.
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 aprepitant. Granisetron is a first-line antiemetic for CINV in chemotherapy. Dexamethasone at 50% dosing is effective in reducing nausea and vomiting. Aprepitant, a neurokinin-1 receptor antagonist, is recommended for moderate to high emetogenic chemotherapy regimens like cisplatin. This combination provides a comprehensive approach targeting different pathways involved in CINV. Choice A is incorrect because olanzapine is not typically used in pediatric patients for CINV prevention. Choice B is incorrect as aprepitant is preferred over olanzapine. Choice D is incorrect because excessive dexamethasone dosing can increase the risk of side effects without additional benefit.
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 for correct answer (C - MYH9): 1. MYH9 gene encodes for non-muscle myosin heavy chain IIA. 2. Mutations in MYH9 gene are associated with May-Hegglin anomaly, characterized by thrombocytopenia without bleeding tendency. 3. Additionally, MYH9-related disorders can present with sensorineural hearing loss. 4. Given the patient's thrombocytopenia and hearing loss, MYH9 gene is the most likely culprit. Summary of incorrect choices: A: NBEAL2 - Associated with gray platelet syndrome, not consistent with patient's presentation. B: GP-1Ba - Glycoprotein Ib-IX-V complex gene, not linked to the symptoms described. D: Deletions of long arm of chromosome 11 - Not specific to thrombocytopenia and hearing loss in this context.
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. Platinum-associated hearing loss is due to the destruction of the cochlear hair cells. Platinum-based chemotherapy agents can cause ototoxicity, leading to sensorineural hearing loss by damaging the hair cells in the cochlea. This type of hearing loss affects the ability to hear high-frequency sounds first. Low-frequency volumes are typically preserved. Older age at exposure does not increase the risk of platinum-associated hearing loss. Conductive hearing loss is not typically associated with platinum chemotherapy. In summary, the correct answer is D because platinum-associated hearing loss affects the cochlear hair cells, leading to sensorineural hearing loss predominantly in high-frequency sounds.
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: Give one more course of intensification chemotherapy and then perform a matched unrelated donor HSCT. In this scenario, the patient achieved morphologic remission and MRD-negative status after the second course of chemotherapy. Performing a matched unrelated donor HSCT can provide the best chance for long-term disease control and potential cure by replacing the patient's hematopoietic system with healthy donor cells, reducing the risk of relapse. This approach combines the benefits of achieving remission with chemotherapy and the potentially curative effects of allogeneic HSCT. The other choices are suboptimal: A may lead to excessive toxicity, B may not be as effective in preventing relapse, and D may not be as curative as HSCT in this high-risk case.