ATI RN
The Hematologic System ATI Questions
Question 1 of 5
An 18-year old male patient presents with bruising, fatigue, and diffuse extremity pain. He is noted to be tachypneic and hypoxic and has a diffuse interstitial infiltrate on chest x-ray. CBC reveals a WBC count of 285,000/mm3 (85% myeloblasts, with monocytic morphology), hemoglobin of 7.9 g/dL, and platelet count of 36,000/mm3. What is the most likely cause of the infiltrate and respiratory symptoms and the most appropriate initial treatment?
Correct Answer: B
Rationale: The correct answer is B: Hyperleukocytosis; leukapheresis or manual exchange transfusion and initiation of induction chemotherapy. In this scenario, the patient's symptoms and lab findings are consistent with acute myeloid leukemia (AML) with leukostasis, causing hypoxia and interstitial infiltrates. Leukapheresis or manual exchange transfusion is crucial to rapidly reduce the high white blood cell count, which can help alleviate symptoms and prevent complications like tissue hypoxia. Initiation of induction chemotherapy is also essential for long-term management of AML. Rationale for why other choices are incorrect: A: Hyperleukocytosis alone without leukostasis does not typically require immediate leukoreduction, as in this case. Induction chemotherapy should be initiated promptly to address the underlying AML. C: COVID-19 infection would not typically present with such profound leukocytosis and monocytic morphology. Convalescent plasma and prednisone are not
Question 2 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 3 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 4 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 5 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.