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. DICER-1 gene mutations can lead to the development of Sertoli-Leydig cell tumors, which can present with these symptoms in adolescent girls. Li-Fraumeni syndrome (A) is characterized by a predisposition to various cancers but not specifically ovarian tumors. Turner syndrome (C) is associated with ovarian dysgenesis leading to primary amenorrhea, not secondary amenorrhea and virilization. Beckwith-Wiedemann syndrome (D) is a genetic overgrowth syndrome with a risk of embryonal tumors but not typically ovarian masses with virilization.
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 because the child is showing signs of vincristine-induced neurotoxicity, which can manifest as droopy eyelids (ptosis) and hoarse cry. Holding vincristine and reevaluating weekly allows for monitoring of symptoms. If resolved, vincristine can be resumed with a dose reduction to prevent further neurotoxicity. Dactinomycin and cyclophosphamide are continued to maintain treatment efficacy. Continuing full-dose vincristine (choice A) can worsen neurotoxicity. With rhabdomyosarcoma responding to the current regimen, stopping all chemotherapy (choice B) is not appropriate. Discontinuing vincristine permanently (choice D) may compromise the treatment plan.
Question 3 of 5
Which of the following is a key feature of Factor XIII?
Correct Answer: A
Rationale: Factor XIII has a long half-life of about 10 days, allowing it to play a crucial role in stabilizing fibrin clots. This extended lifespan contributes to the maintenance of clot integrity over time. The other choices are incorrect because Factor XIII is not an activator of thrombin (B), its levels are low in newborns and gradually increase (C), and it is not part of the contact activation system (D).
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 patients with severe factor XI deficiency, like this 16-year-old female, fresh frozen plasma can provide factor XI replacement and improve hemostasis. The dose of 20 mL/kg is appropriate to achieve a factor XI level of around 20%, which is sufficient for hemostasis during surgery. Now, let's analyze the other choices: A: Cryoprecipitate (five units), which will likely raise her factor XI level to 20% - Cryoprecipitate contains factor VIII and fibrinogen, but the amount of factor XI in cryoprecipitate is minimal and may not significantly raise factor XI levels to the required 20%. B: Factor XI concentrate (20 units/kg), which will raise her factor XI level to 20% - While factor XI concentrate is
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 is a symptom more commonly associated with musculoskeletal issues rather than pulmonary embolism. Pulmonary embolism typically presents with symptoms such as chest pain, shortness of breath, and sometimes cough. Fever is not a typical symptom of pulmonary embolism. Shortness of breath is a common symptom but not specific enough to substantially decrease suspicion for pulmonary embolism.