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 classic features of NF1. NF1 is associated with multiple neurocutaneous manifestations, including café-au-lait spots, neurofibromas, and optic pathway gliomas. Li Fraumeni syndrome (B) is a cancer predisposition syndrome characterized by a high risk of developing various cancers, not typically associated with optic pathway gliomas or skin manifestations. Tuberous sclerosis (C) presents with features like facial angiofibromas, cortical tubers, and cardiac rhabdomyomas, not axillary freckling or optic pathway gliomas. Cystic Fibrosis (D) 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: Step-by-step rationale for why the correct answer is D, Nephroblastoma: 1. The child has a cancer predisposition syndrome, likely Beckwith-Wiedemann syndrome (BWS). 2. BWS is associated with an increased risk of developing Wilms tumor (nephroblastoma). 3. The large tongue, omphalocele, and overgrowth placing the child at the 95th percentile for height and weight are all characteristic features of BWS. 4. Therefore, the patient is most at risk of developing nephroblastoma. Summary of why the other choices are incorrect: A: Pleuropulmonary blastoma - Not typically associated with BWS or the described clinical features. B: Hepatocellular carcinoma - Not commonly linked to BWS or the clinical presentation provided. C: Cystic nephroma - Not a typical tumor associated with BWS or the clinical findings described in the patient.
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 case, the child is likely suffering from Glanzmann thrombasthenia, a rare inherited platelet disorder where platelets lack the ability to bind to fibrinogen. This results in impaired platelet aggregation and clot formation, leading to bleeding tendencies. The other options (A, C, D) are not affected in Glanzmann thrombasthenia. Platelets interact normally with ADP (A), von Willebrand factor (C), and Platelet factor 4 (D) in this disorder.
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 staging, CNS involvement is rare at initial presentation. Therefore, routine CSF analysis is not recommended unless there are specific neurological symptoms suggestive of CNS disease. A lumbar puncture carries risks of complications and should not be performed routinely. Choices A, B, and C are appropriate for staging in Hodgkin lymphoma to evaluate for distant metastases and characterize disease extent.
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: Step 1: The patient's pain is described as burning, tingling, and shooting in her left leg, which suggests neuropathic pain. Step 2: The pain worsens with hot showers, indicating sensitivity to temperature changes, a common feature of neuropathic pain. Step 3: Gabapentin is a first-line medication for neuropathic pain, as it stabilizes nerve cells and reduces abnormal pain signaling. Step 4: Starting low and titrating upward on gabapentin dose helps minimize side effects and optimize pain relief. Step 5: Methadone (Choice A) is not the best option for neuropathic pain. Increasing immediate release oxycodone (Choice B) may lead to opioid tolerance. Amitriptyline (Choice C) may help neuropathic pain but is not as specific as gabapentin.