ATI RN
The Hematologic System ATI Questions
Question 1 of 5
You have been asked to see a 15-year-old girl who is being referred for evaluation of an ovarian mass. Her history is also significant for secondary amenorrhea, and physical examination shows signs of virilization. As you review her family history, what syndrome will you consider?
Correct Answer: B
Rationale: The correct answer is B: DICER-1 syndrome. This syndrome is associated with ovarian tumors, secondary amenorrhea, and signs of virilization due to androgen-secreting tumors. Li-Fraumeni syndrome (A) is characterized by multiple primary cancers but not specifically ovarian masses. Turner syndrome (C) presents with ovarian dysgenesis and primary amenorrhea. Beckwith-Wiedemann syndrome (D) is associated with overgrowth and abdominal wall defects, not ovarian masses. In this case, the presence of ovarian mass, secondary amenorrhea, and virilization point towards DICER-1 syndrome.
Question 2 of 5
A 2-month-old girl is found to have a small, hard mass on her scalp. The mass increases in size over the next 4 weeks. A biopsy is performed that confirms a diagnosis of embryonal rhabdomyosarcoma. You initiate chemotherapy with vincristine, dactinomycin, and cyclophosphamide. The child presents to clinic for day 1 of cycle 3 of chemotherapy, and the mass on her scalp is smaller. She is afebrile, absolute neutrophil count is 1,405 cells/mcL, platelet count is 154,000/mcL, and total bilirubin is 0.8 mg/dL. Her mother reports she looks very tired because her eyelids have been 'very droopy,' and she thinks she has a sore throat because her cry is hoarse. Her last bowel movement was 2 days ago. What is the most appropriate chemotherapy plan?
Correct Answer: C
Rationale: The correct answer is C: Administer dactinomycin and cyclophosphamide but hold the vincristine and reevaluate weekly. If the ptosis and hoarse cry resolve, vincristine can be resumed with a dose reduction and, if tolerated, re-escalated to the full dose in the future. Rationale: 1. Ptosis and hoarse cry are symptoms of vincristine-induced neurotoxicity. 2. Holding vincristine allows for resolution of these side effects. 3. Reevaluating weekly ensures close monitoring of symptoms. 4. If symptoms resolve, vincristine can be cautiously reintroduced with a dose reduction to prevent further neurotoxicity. Summary: A: Continuing all chemotherapy may worsen neurotoxicity. B: Withholding all chemotherapy is not appropriate if two agents are effective. D: Discontinuing vincristine permanently may limit treatment options.
Question 3 of 5
Which of the following is a key feature of Factor XIII?
Correct Answer: A
Rationale: The correct answer is A: Its half-life is about 10 days. Factor XIII has a long half-life due to its stable tetrameric structure, allowing it to circulate in the blood for about 10-14 days. This extended half-life is crucial for its role in stabilizing fibrin clots. Explanation for why other choices are incorrect: B: Factor XIII is not an activator of thrombin; rather, it crosslinks fibrin strands to strengthen clots. C: Factor XIII levels are low in newborns and gradually increase during the first few months of life. D: Factor XIII is not part of the contact activation system, which involves factors XII, XI, and prekallikrein.
Question 4 of 5
A 16-year-old female patient with severe factor XI deficiency presents with acute appendicitis and requires urgent surgery. You are called by the surgeon, who wants to know what, if any, blood products or treatments are required to reduce the risk of perioperative bleeding. The patient weighs 62 kg. What should you tell him to administer?
Correct Answer: C
Rationale: The correct answer is C: Fresh frozen plasma (20 mL/kg), which will raise her factor XI level to 20%. Fresh frozen plasma contains various clotting factors, including factor XI. In a patient with severe factor XI deficiency, administering fresh frozen plasma can increase factor XI levels and help reduce the risk of perioperative bleeding. The dose of 20 mL/kg is appropriate for this patient's weight of 62 kg. Cryoprecipitate (choice A) may contain factor XI but is not the optimal choice for raising factor XI levels specifically. Factor XI concentrate (choice B) would be ideal but is not commonly available, making fresh frozen plasma a more practical option. Prothrombin complex concentrate (choice D) primarily contains factors II, VII, IX, and X, but not factor XI, so it would not effectively raise factor XI levels in this patient.
Question 5 of 5
A 16-year-old female presents to the emergency room with a new complaint of chest pain. When performing a review of systems and physical examination, which of the following would substantially decrease your suspicion for a diagnosis of pulmonary embolism?
Correct Answer: C
Rationale: The correct answer is C: Rib tenderness. Rib tenderness would decrease suspicion for pulmonary embolism because it suggests a musculoskeletal origin of chest pain rather than a vascular one. Pulmonary embolism typically presents with symptoms such as shortness of breath, pleuritic chest pain, and sometimes cough, but rib tenderness is not a common finding in this condition. Fever is non-specific and can be present in various conditions, including pulmonary embolism. Shortness of breath is a key symptom of pulmonary embolism and would increase suspicion for this diagnosis.