ATI RN
The Hematologic System ATI Questions
Question 1 of 5
Several gene mutations have been associated with juvenile myelomonocytic leukemia (JMML), and they may or may not have prognostic implications. A gene expression–based classification system has been found to be an independent predictor of clinical outcome in these patients. What is the disease signature that predicts a poor outcome?
Correct Answer: B
Rationale: The correct answer is B: Acute myeloid leukemia-like. This is because JMML shares similarities with acute myeloid leukemia in terms of aggressive progression and poor outcomes. Children with JMML who exhibit an acute myeloid leukemia-like gene expression signature have been shown to have a worse prognosis compared to those with other gene expression profiles. The other choices (A, C, D) are incorrect because tyrosine kinase inhibitors are not directly related to predicting clinical outcomes in JMML, chronic myeloid leukemia-like gene expression profile does not necessarily predict poor outcomes in JMML, and BRAF pathway abnormalities are not specifically associated with predicting poor outcomes in JMML.
Question 2 of 5
You are seeing a 12-year-old female who presented to the emergency department with the sudden onset of severe abdominal pain. Imaging that was obtained to rule out appendicitis revealed a mass adjacent to the bladder. The mass was surgically resected, and pathology demonstrated a paraganglioma. Which of the studies below would be most useful to determine disease stage for this patient?
Correct Answer: D
Rationale: The correct answer is D: Ga 68-DOTATATE PET/CT. This imaging study is the most useful for determining the disease stage in a patient with paraganglioma. Paragangliomas are neuroendocrine tumors that express somatostatin receptors, which can be detected using Ga 68-DOTATATE PET/CT. This imaging modality helps to localize primary and metastatic lesions, as well as assess disease extent and stage. Now let's analyze the other options: A: Bone Scan - Not useful for determining disease stage in paraganglioma. B: Lumbar puncture for cerebrospinal fluid cytology - Not indicated for staging paraganglioma. C: Bone marrow aspirate and biopsy - Not specific for staging paraganglioma. In summary, Ga 68-DOTATATE PET/CT is the most appropriate study for determining disease stage in a patient with paraganglioma due to its
Question 3 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 4 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 5 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