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. This patient most likely has Beckwith-Wiedemann Syndrome (BWS) due to the large tongue (macroglossia) and omphalocele. BWS is associated with an increased risk of nephroblastoma (Wilms tumor). The 95th percentile for height and weight is also a characteristic feature of BWS. Ultrasound and laboratory evaluation are appropriate monitoring tools for nephroblastoma in this patient due to the increased risk associated with BWS. A: Pleuropulmonary blastoma is more commonly associated with DICER1 syndrome, not BWS. B: Hepatocellular carcinoma is not commonly associated with BWS. C: Cystic nephroma is not typically associated with BWS, and nephroblastoma is a more common tumor in this context.
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 this case, the key features to consider are the patient's age (9 months old), large liver mass with lung metastases, and elevated alpha-fetoprotein level. Small cell undifferentiated hepatoblastoma is commonly seen in infants, presents as a large liver mass, and frequently metastasizes to the lungs. The alpha-fetoprotein level in this case is also elevated, which is typical for hepatoblastoma. Pure fetal histology hepatoblastoma (A) is less common in older infants, and the presence of lung metastases is not typical. Embryonal sarcoma (B) typically presents as a solitary mass without metastases. Fibrolamellar hepatocellular carcinoma (C) is rare in infants and does not typically present with elevated alpha-fetoprotein levels. Thus, based on the clinical presentation and imaging findings, small cell undifferentiated hepatob
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 increased risk of disease recurrence. This mutation disrupts chromatin remodeling, leading to genomic instability and aggressive tumor behavior. ETV6-NTRK3 gene fusion (Choice A) is more commonly associated with pediatric mesenchymal tumors. PTPN11 mutation (Choice B) is typically found in Noonan syndrome and other malignancies, not neuroblastoma. WT1 mutation (Choice D) is more commonly seen in Wilms tumor, not neuroblastoma. In summary, the ATRX mutation is the most likely molecular aberration detected in this case based on the clinical presentation and outcomes of the patient.
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 sequence is appropriate because methotrexate can interfere with the activity of L-asparaginase if given concurrently, affecting the efficacy of both drugs. Administering L-asparaginase immediately after the methotrexate infusion allows for optimal therapeutic effects of both medications without compromising their individual actions. Choice A (Administer L-asparaginase during the methotrexate infusion) is incorrect because it may lead to drug interactions and reduced effectiveness of both drugs. Choice C (Administer both drugs at the same time) is incorrect for the same reason as choice A. Choice D (Administer methotrexate 24 hours after the asparaginase) is incorrect as it does not follow the optimal timing for these medications during treatment.
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: The correct answer is D: Thrombomodulin. Thrombomodulin binds to thrombin and changes its function from procoagulant to anticoagulant by activating protein C. This leads to the downregulation of fibrinogen formation. Protein C (choice A) and its cofactor Protein S (choice B) are involved in the anticoagulant pathway but do not directly alter thrombin's function. Antithrombin (choice C) inhibits thrombin and other coagulation factors, but it doesn't specifically change thrombin's function from procoagulant to anticoagulant.