ATI RN
ATI Hematologic System Quizlet 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 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 2 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 3 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.
Question 4 of 5
The patient is a 2-month-old boy who presented with a skin abscess and is febrile. On exam, he is noted to have silvery hair and hypopigmented skin. A CBC shows a leukocyte count of 3.4 K/mcL with 10% neutrophils. What does the abnormality on the peripheral smear suggest?
Correct Answer: A
Rationale: The correct answer is A: Abnormal lysosomal biogenesis. The patient's presentation of silvery hair and hypopigmented skin suggests a lysosomal storage disorder like Chediak-Higashi syndrome. The peripheral smear showing 10% neutrophils with a low leukocyte count indicates impaired neutrophil function due to abnormal lysosomal biogenesis. This affects phagocytosis and intracellular killing of pathogens, leading to recurrent infections and abscess formation. Incorrect choices: B: Abnormal ribosome function - not related to the patient's presentation. C: Abnormal phagocytosis of opsonized particles - the primary issue is with lysosomal biogenesis, not phagocytosis. D: Abnormal mitochondrial activity - does not explain the silvery hair and hypopigmented skin seen in Chediak-Higashi syndrome.
Question 5 of 5
A 19-year old male patient with a history of acute lymphoblastic leukemia, currently 13 years from completion of therapy, presents for a fertility consultation. He is interested in his risk for infertility. Which of the following statements is true?
Correct Answer: A
Rationale: Step 1: Semen analysis is the gold standard for assessing male fertility. Step 2: The patient's history of ALL and completion of therapy make semen analysis relevant. Step 3: Long-term survivors of ALL are at risk for infertility due to treatment effects. Step 4: Semen analysis can provide valuable information on sperm count, motility, and morphology. Summary: - Option A is correct as semen analysis is crucial for assessing male fertility. - Option B is incorrect as alkylator dosages affect both males and females. - Option C is incorrect as sperm cryopreservation should ideally be offered at diagnosis. - Option D is incorrect as infertility risk may be higher than testosterone deficiency in this case.