ATI RN
The Hematologic System ATI Questions
Question 1 of 5
A 4-year-old child with acute lymphoblastic leukemia is receiving high-dose methotrexate during interim maintenance. He receives ondansetron and арrepitant during his stay, which control his nausea and vomiting well. These medications work by inhibiting signaling in which part of the brain?
Correct Answer: E
Rationale: The correct answer is D: Vomiting center. Ondansetron and aprepitant are antiemetic drugs that work by targeting the vomiting center in the brainstem. The vomiting center coordinates the vomiting reflex in response to various stimuli, including chemotherapy drugs like methotrexate. By inhibiting signaling in the vomiting center, these medications effectively prevent nausea and vomiting. The other choices (A: Vestibular system, B: Cerebral cortex, C: Hypothalamus) are not directly involved in the control of nausea and vomiting in response to chemotherapy.
Question 2 of 5
An 18-year old male patient with acute lymphoblastic leukemia recently started maintenance therapy and is complaining of increased hip pain. The pain increases during weight-bearing activity; however, it occasionally hurts at night as well. His CBCd is normal. Which of the following risk factors is most commonly associated with this process?
Correct Answer: D
Rationale: The correct answer is D: Dexamethasone exposure. Dexamethasone is a corticosteroid commonly used in the treatment of acute lymphoblastic leukemia. It can lead to avascular necrosis of the hip, causing hip pain during weight-bearing activities. This is due to its negative impact on bone health and blood supply to the hip joint. Other choices are incorrect because younger age at diagnosis is not a risk factor for avascular necrosis, non-White race and low body-mass index are not directly associated with this process.
Question 3 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 4 of 5
You are consulting on a 10-year-old male with severe persistent neutropenia, a history of recurrent infections, and warts. The rest of the peripheral blood count is normal. His mother also has neutropenia. Bone marrow examination shows a hypercellular marrow and retained myeloid cells with vacuolated cytoplasm. There are no abnormalities in the red cells or platelet precursors. Cytogenetics are 46XY. You start granulocyte colony stimulating factor therapy and the neutrophil count increases. A mutation in which of the following genes is most likely to have caused this familial inherited bone marrow failure syndrome?
Correct Answer: A
Rationale: The correct answer is A: CXCR4. In this case, the familial inherited bone marrow failure syndrome with severe neutropenia, recurrent infections, and warts suggests WHIM syndrome. WHIM syndrome is caused by a gain-of-function mutation in the CXCR4 gene, leading to impaired neutrophil trafficking. The symptoms and bone marrow findings in this patient align with WHIM syndrome. Choice B (ELANE) is associated with cyclic neutropenia, not WHIM syndrome. Choice C (GATA 2) is linked to familial myelodysplastic syndromes and acute myeloid leukemia, not WHIM syndrome. Choice D (Mitochondrial DNA) is not associated with WHIM syndrome. Therefore, the mutation in CXCR4 is the most likely cause of the familial inherited bone marrow failure syndrome in this patient.
Question 5 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.