ATI RN
ATI Hematologic System Quizlet Questions
Question 1 of 5
A 6-year-old boy is noted to have worsening visual acuity on serial eye exams. Recent MRI of his brain and orbits reveals an optic chiasm mass, most consistent with an optic pathway glioma. Upon further examination, the physician notes axillary freckling and numerous “spots†on the patient's skin. This patient most likely has a which of the following genetic disorders?
Correct Answer: A
Rationale: The correct answer is A: Neurofibromatosis type-1 (NF1). This is because the patient presents with optic pathway glioma, axillary freckling, and numerous skin spots, which are characteristic features of NF1. NF1 is caused by a mutation in the NF1 gene on chromosome 17, leading to the development of various tumors. Li Fraumeni syndrome is associated with multiple cancers but not specifically with optic pathway glioma or skin findings. Tuberous sclerosis presents with facial angiofibromas, cortical tubers, and subependymal nodules, not optic pathway glioma. Cystic fibrosis is a genetic disorder affecting the lungs and digestive system, not associated with the symptoms described in the patient.
Question 2 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 likely has Beckwith-Wiedemann syndrome based on the large tongue (macroglossia) and omphalocele. Beckwith-Wiedemann syndrome is associated with an increased risk of developing nephroblastoma (Wilms tumor). The 95th percentile for height and weight is also a common feature of this syndrome. Pleuropulmonary blastoma (choice A) is more commonly seen in patients with DICER1 mutations. Hepatocellular carcinoma (choice B) is not typically associated with Beckwith-Wiedemann syndrome. Cystic nephroma (choice C) is more commonly seen in patients with DICER1 mutations, not Beckwith-Wiedemann syndrome.
Question 3 of 5
An 18-month-old boy, whose parents are first cousins, is referred to you because of a significant episode of epistaxis. The parents report that the child had bleeding after circumcision and large hematomas with immunizations. Platelet aggregation studies show the following: This child's platelets are unable to interact with which of the following?
Correct Answer: B
Rationale: The correct answer is B: Fibrinogen. In this scenario, the child likely has Glanzmann thrombasthenia, a rare inherited platelet disorder where platelets lack functional integrin αIIbβ3, preventing binding to fibrinogen. This results in impaired platelet aggregation and clot formation, leading to bleeding issues. Choices A, C, and D are incorrect because platelet aggregation studies would typically show normal interactions with ADP, von Willebrand factor, and Platelet factor 4 in Glanzmann thrombasthenia.
Question 4 of 5
A 17-year-old female presents with cervical adenopathy and a history of daily fevers and drenching night sweats. A biopsy is performed and reveals classic Hodgkin lymphoma. Which of the following is least appropriate as part of the staging workup?
Correct Answer: D
Rationale: The correct answer is D, Lumbar puncture and cerebrospinal fluid (CSF) analysis. In Hodgkin lymphoma, central nervous system involvement is rare at presentation, making routine CSF analysis unnecessary. Staging workup typically includes imaging studies (A, B, C) to assess disease extent and involvement of distant organs. CSF analysis is reserved for cases with neurological symptoms or signs suggestive of CNS involvement. Therefore, in this case, the least appropriate option for staging workup is D.
Question 5 of 5
A 16-year-old patient with a left-side pelvic osteosarcoma is taking extended release oxycodone twice daily as well as immediate release oxycodone for breakthrough pain approximately 2 or 3 times per day. She describes her pain as burning, tingling, and shooting in her left leg. Her pain worsens with hot showers. Her most recent EKG has a QTc of 495. What would be the best strategy to manage her pain?
Correct Answer: D
Rationale: The correct answer is D: Add gabapentin three times daily. Gabapentin is a first-line option for neuropathic pain, which is likely the cause of the patient's burning, tingling, and shooting leg pain. The worsening of pain with hot showers is also characteristic of neuropathic pain. Gabapentin's mechanism of action in reducing neuropathic pain makes it a suitable choice for this patient. Switching to methadone (A) may not directly address the neuropathic pain component. Recommending more frequent use of immediate release oxycodone (B) may lead to increased risk of opioid-related adverse effects. While amitriptyline (C) is also used for neuropathic pain, starting with gabapentin is more appropriate given the patient's current opioid regimen and EKG findings.