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. In JMML, a disease signature resembling acute myeloid leukemia (AML) has been associated with a poor outcome. This signature includes features such as increased blasts, abnormal karyotypes, and mutations in genes like NRAS and KRAS. AML-like JMML cases often have aggressive disease progression and poorer response to treatment. Tyrosine kinase inhibitors (choice A) are not typically used in JMML treatment. Chronic myeloid leukemia-like (choice C) is not associated with a poor outcome in JMML. BRAF pathway abnormalities (choice D) may be present in some cases of JMML but are not the primary disease signature predicting poor outcomes.
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. Paragangliomas are neuroendocrine tumors that can secrete catecholamines and have a high expression of somatostatin receptors. Ga 68-DOTATATE PET/CT is the most appropriate imaging study to determine disease stage in patients with paragangliomas because it can detect the somatostatin receptor expression in these tumors, aiding in localization, staging, and treatment planning. A: Bone scan is not the most useful study for determining disease stage in paraganglioma. B: Lumbar puncture for cerebrospinal fluid cytology is not relevant for staging paraganglioma. C: Bone marrow aspirate and biopsy are not the most appropriate studies for staging paraganglioma.
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: 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 4 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 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 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.