ATI RN
ATI Hematologic System Quizlet Questions
Question 1 of 5
You are consulted on a 4-year-old girl who is newly diagnosed with standard-risk pre-B acute lymphoblastic leukemia. After reviewing her previous complete blood examinations, you note she has had a platelet count ranging from 80,000 to 100,000 cells/mcL over the past 2 years. Her father mentions that he has also been told he has mild thrombocytopenia. You suspect the child may have a cancer predisposition syndrome. Which sample should you send for analysis, and which gene is most likely implicated?
Correct Answer: B
Rationale: The correct answer is B: Skin fibroblasts to evaluate the ETV6 gene. In this scenario, the presence of mild thrombocytopenia in both the father and the child raises suspicion of a genetic predisposition. ETV6 gene mutations are commonly associated with inherited thrombocytopenia and predisposition to leukemia. Skin fibroblasts are ideal for genetic testing due to their stable genetic material. Choice A is incorrect as RUNX1 gene mutations are linked to familial platelet disorder with predisposition to acute myeloid leukemia, not pre-B acute lymphoblastic leukemia. Choices C and D are incorrect as buccal swabs may not provide sufficient genetic material for comprehensive analysis.
Question 2 of 5
A 3-year-old boy is referred to you for evaluation of right leukocoria. Funduscopic examination under anesthesia reveals a large amelanotic mass occupying more than two-thirds of the vitreous space in his right eye, with massive retinal detachment, consistent with group E retinoblastoma. The left eye is normal. An MRI confirms the funduscopic findings and shows no extraocular disease. What is the most appropriate next step in the management of this child's disease?
Correct Answer: A
Rationale: The most appropriate next step in the management of the child's retinoblastoma is enucleation (choice A). Enucleation, the surgical removal of the affected eye, is indicated when there is a large intraocular tumor with extensive retinal detachment, as in this case. Enucleation can provide local control of the disease and prevent systemic spread. It is considered the standard treatment for advanced retinoblastoma to prevent metastasis. Systemic chemotherapy (choice B) is not the first-line treatment for advanced retinoblastoma with massive intraocular involvement. Brachytherapy (choice C) involves the placement of a radioactive source near the tumor, which may not be effective in this case of extensive intraocular disease. Needle biopsy (choice D) is not recommended as the primary management strategy due to the risk of seeding tumor cells outside the eye. Therefore, enucleation is the most appropriate next step in this scenario.
Question 3 of 5
Your patient with relapsed high-risk neuroblastoma returns to your care after travelling to an outside institution for [131]I-MIBG therapy. In the weeks following [131]I-MIBG therapy, what adverse events directly attributable to this therapy will the patient most likely encounter?
Correct Answer: A
Rationale: Rationale for Correct Answer (A): Myelosuppression requiring growth factor and blood product support is the most likely adverse event following [131]I-MIBG therapy due to its impact on bone marrow. The therapy targets neuroblastoma cells, but can also affect normal bone marrow function, leading to myelosuppression. Growth factors and blood products are often needed to support hematopoiesis. Summary of Incorrect Answers: B: Severe mucositis: Not a common adverse event associated with [131]I-MIBG therapy, as it primarily affects the bone marrow. C: Hemorrhagic cystitis: Not directly related to [131]I-MIBG therapy, which does not typically cause bladder toxicity. D: Symptomatic hypothyroidism: While [131]I-MIBG therapy can affect thyroid function, symptomatic hypothyroidism is not the most likely adverse event following this therapy.
Question 4 of 5
A 12-year-old patient with localized osteosarcoma is being treated with cisplatin, doxorubicin, and high-dose methotrexate. The pain at his primary site rapidly resolves after initiation of chemotherapy. After tumor resection, pathology reveals the tumor was greater than 95% necrotic. You want to continue cisplatin, doxorubicin, and high-dose methotrexate. Which of the following is the best answer regarding the evaluations that should be performed to monitor for toxicity in patients receiving cisplatin, doxorubicin, and high-dose methotrexate?
Correct Answer: C
Rationale: The correct answer is C because it includes essential evaluations to monitor for toxicity associated with cisplatin, doxorubicin, and high-dose methotrexate. 1. Complete blood count: To monitor for potential bone marrow suppression caused by chemotherapy drugs. 2. Creatinine: To assess renal function, as cisplatin can cause nephrotoxicity. 3. Serum magnesium: Monitoring for hypomagnesemia, a common side effect of cisplatin. 4. Audiogram: To screen for hearing loss, a known side effect of cisplatin. 5. Echocardiogram: To evaluate for cardiotoxicity, especially with doxorubicin known to cause cardiomyopathy. Other choices are incorrect: A: Does not include serum magnesium, audiogram, and echocardiogram, which are important for monitoring toxicity. B: While electrolytes and EKG are relevant, it lacks creatinine, serum magnesium, and other crucial tests. D:
Question 5 of 5
Which of the following characteristics are similar with respect to Factor VIII and von Willebrand factor (vWF)?
Correct Answer: C
Rationale: Rationale: - Factor VIII and vWF are present in normal to high amounts in newborns due to the physiological immaturity of the hemostatic system. - Choice A is incorrect as vWF is mainly produced in endothelial cells while Factor VIII is produced in both endothelial cells and liver. - Choice B is incorrect as Factor VIII is activated by thrombin, but vWF is not. - Choice D is incorrect as vWF is stored in Weibel-Palade bodies, but Factor VIII is not.