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. The patient's presentation with tachypnea, hypoxia, and diffuse interstitial infiltrate on chest x-ray suggests leukostasis syndrome due to hyperleukocytosis. The extremely high WBC count of 285,000/mm3 with myeloblasts indicates acute myeloid leukemia. Leukapheresis or manual exchange transfusion is needed to rapidly reduce the number of leukemic blasts in circulation to prevent complications like tissue hypoxia. Initiation of induction chemotherapy is essential for long-term management of AML. Choice A is incorrect because induction chemotherapy alone may not rapidly reduce the WBC count in cases of leukostasis. Choice C is incorrect as the patient's clinical scenario is not consistent with COVID-19 infection, and convalescent plasma is not indicated for leukostasis. Choice D is incorrect as the patient's symptoms are not typical for pneumococcal pneumonia, and vancomycin is not the initial
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. 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 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. 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 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. 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 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 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.