ATI RN
ATI Hematologic System Test Questions
Question 1 of 5
You receive a phone call from a community pediatrician who is caring for a 2-year-old toddler with a cancer predisposition syndrome. The pediatrician describes a child at the 95th percentile for height and weight with a history of corrective oral surgery to reduce a large tongue and a history of an omphalocele in infancy. The pediatrician is currently performing ultrasound of the abdomen and laboratory evaluation for this patient every 3 months. Which tumor is this patient most at risk of developing?
Correct Answer: D
Rationale: The correct answer is D: Nephroblastoma (Wilms tumor). This patient likely has Beckwith-Wiedemann syndrome given the features of overgrowth, macroglossia, and omphalocele. Beckwith-Wiedemann syndrome is associated with an increased risk of Wilms tumor. Wilms tumor is the most common renal malignancy in childhood. Monitoring for Wilms tumor with ultrasound and laboratory evaluations is appropriate due to the increased risk in this patient population. Choice A: Pleuropulmonary blastoma is a rare lung tumor more commonly seen in children under 2 years old. It is not typically associated with Beckwith-Wiedemann syndrome. Choice B: Hepatocellular carcinoma is a primary liver cancer more commonly seen in adults, not children with Beckwith-Wiedemann syndrome. Choice C: Cystic nephroma is a benign kidney tumor typically seen in young children, but it is not associated with Beckwith-Wiedemann syndrome nor is it malignant
Question 2 of 5
A 9-month-old boy has been referred to you for the evaluation of an enlarged abdomen. Imaging studies show a large liver mass (PRETEXT III). Alfa-fetoprotein is 98 ng/mL, and a CT scan of the lungs show bilateral lung metastases. A needle biopsy is performed, and you are planning to review the specimen with the pathologist. Which of the following diagnoses are you suspecting?
Correct Answer: D
Rationale: The correct answer is D: Small cell undifferentiated hepatoblastoma. In hepatoblastoma, the presence of lung metastases indicates a high-risk tumor. The small cell undifferentiated subtype is more aggressive with a poorer prognosis compared to other subtypes. The AFP level is lower than typically seen in hepatoblastoma, but still within the range for this diagnosis. Pure fetal histology hepatoblastoma (choice A) is less common and usually associated with a better prognosis. Embryonal sarcoma of the liver (choice B) is a distinct entity with different histological features. Fibrolamellar hepatocellular carcinoma (choice C) typically occurs in older children and has a different imaging appearance.
Question 3 of 5
An otherwise healthy 18-year-old female is diagnosed with high-risk neuroblastoma after presenting with fatigue and bony pain. Imaging findings demonstrate a left adrenal mass with multiple osseous metastases. She successfully completes standard therapy for high-risk neuroblastoma, but experiences several episodes of disease recurrence and ultimately dies of her disease 10 years after her initial diagnosis. During her treatment, her tumor was sent for molecular analysis. Of the following, what molecular aberration was most likely to have been detected?
Correct Answer: C
Rationale: The correct answer is C: ATRX mutation. In neuroblastoma, ATRX mutations are associated with poor prognosis and high-risk disease. ATRX gene mutations are commonly found in cases with aggressive behavior and poor outcomes, such as in this case where the patient experienced disease recurrence and ultimately died. ATRX mutations are linked to chromosomal instability and telomere dysfunction, which can contribute to tumor progression and resistance to therapy. A: ETV6-NTRK3 gene fusion is more commonly associated with infantile fibrosarcoma and secretory breast carcinoma, not neuroblastoma. B: PTPN11 mutations are typically seen in juvenile myelomonocytic leukemia and Noonan syndrome, not neuroblastoma. D: WT1 mutations are more commonly found in Wilms tumor and acute myeloid leukemia, not neuroblastoma. In summary, the ATRX mutation is the most likely molecular aberration detected in this patient with high-risk neuroblast
Question 4 of 5
A 9-year-old boy is being treated for standard-risk acute lymphoblastic leukemia. His treatment protocol calls for administration of intravenous methotrexate and intramuscular L-asparaginase during interim maintenance chemotherapy. What is the most appropriate sequence of drug administration?
Correct Answer: B
Rationale: The correct answer is B: Administer L-asparaginase immediately after the methotrexate infusion. This is the most appropriate sequence because methotrexate can impair the activity of L-asparaginase if administered together. By waiting to administer L-asparaginase after the methotrexate infusion, the efficacy of both drugs is maximized without interference. Administering L-asparaginase during the methotrexate infusion (Choice A) would lead to decreased effectiveness of L-asparaginase. Administering both drugs at the same time (Choice C) is not recommended due to potential drug interactions. Administering methotrexate 24 hours after the asparaginase (Choice D) does not optimize the synergistic effects of the drugs during interim maintenance chemotherapy.
Question 5 of 5
Which of the following alters the function of thrombin from a procoagulant protein to one that downregulates the formation of fibrinogen?
Correct Answer: D
Rationale: Thrombomodulin alters thrombin's function by binding to it and activating protein C. Activated protein C then inhibits factors Va and VIIIa, which are essential for the formation of fibrinogen. Protein C (choice A) also inhibits coagulation, but it doesn't directly alter thrombin's function. Protein S (choice B) enhances protein C's activity but doesn't directly affect thrombin. Antithrombin (choice C) inhibits thrombin and other coagulation factors, but it doesn't specifically alter thrombin's function to downregulate fibrinogen formation.