ATI RN
Hematological System Questions
Question 1 of 5
A 3-year-old nonsyndromic, well-appearing male with no significant past medical history presents with an abdominal mass palpated by his mother when giving him a bath. CT imaging reveals a 9-cm right renal mass without involvement of the inferior vena cava (IVC) and no evidence of tumor thrombus by ultrasound. The left kidney appears normal, and there is no imaging evidence of tumor rupture or adherence to surrounding organs. There are diffuse, bilateral pulmonary metastases from which he is asymptomatic with a normal respiratory rate and no supplemental oxygen requirement. Following the National Wilms Tumor Study Group (NWTS)/Children's Oncology Group (COG) approach to pediatric renal tumors, which of the following are appropriate next steps?
Correct Answer: B
Rationale: The correct answer is B: Nephrectomy with lymph node sampling followed by chemotherapy based on histology and stage. This is the appropriate next step following NWTS/COG approach for pediatric renal tumors. Nephrectomy is crucial to remove the primary tumor and obtain tissue for definitive histologic diagnosis. Lymph node sampling helps determine if there is spread to regional nodes. Based on the histology and stage determined post-nephrectomy, appropriate chemotherapy can be initiated tailored to the specific characteristics of the tumor. Explanation of other choices: A: Core biopsy followed by chemotherapy is not recommended as it may not provide sufficient tissue for histologic diagnosis and staging. C: Fine-needle aspiration is not adequate for a definitive diagnosis or staging and may not provide enough tissue for necessary testing. D: Neoadjuvant chemotherapy before surgery is not appropriate in this scenario as the primary tumor needs to be resected for accurate staging and histologic evaluation.
Question 2 of 5
A newborn male has severe bleeding after circumcision, resulting in the need for a blood transfusion. You are called to consult on this child, and you diagnose him with severe hemophilia A. Upon taking a family history, you note that no other family members have hemophilia, other bleeding disorders, or a bleeding diathesis. Which of the following is the most likely outcome of genotyping the Factor VIII gene?
Correct Answer: C
Rationale: The correct answer is C: An inversion mutation in the F8 gene will be identified. In hemophilia A, around 50% of cases are due to large intragenic inversions involving the Factor VIII gene. In this scenario, since there is no family history of hemophilia or other bleeding disorders, the most likely genetic cause is an inversion mutation in the F8 gene. This type of mutation is not dependent on a family history and can occur spontaneously. Missense mutations (choice B) and nonsense mutations (choice D) are less common in hemophilia A and are not the typical genetic abnormalities associated with this condition. Choice A is incorrect because the absence of a family history does not rule out the possibility of a genetic mutation.
Question 3 of 5
A healthy 17-year-old African American male presents with a thrombosis of the right upper extremity. His past medical history is remarkable only for sickle cell trait. The history is negative for recent risk factors for thrombosis (illness, surgery, immobility). He is the pitcher for his high school baseball team. Imaging confirms anatomical compression/narrowing of the right subclavian vein. Which of the following interventions is most likely to decrease this patient's long-term risk of recurrent thrombosis?
Correct Answer: D
Rationale: The correct answer is D: Resection of right first rib. The patient likely has thoracic outlet syndrome (TOS), which is causing compression of the subclavian vein leading to thrombosis. Resection of the right first rib is the definitive treatment for TOS, as it relieves the compression on the vein. This intervention addresses the root cause of the thrombosis, reducing the risk of recurrence. A: Systemic thrombolysis is not indicated in this case as it carries an increased risk of bleeding and may not address the underlying cause of the thrombosis. B: Catheter-directed thrombolysis is not the primary treatment for TOS and may not prevent recurrence. C: Anticoagulation with LMWH does not address the underlying compression of the vein and is not the primary treatment for TOS.
Question 4 of 5
Iron-refractory iron deficiency anemia (IRIDA) is a rare inherited condition characterized by congenital iron deficiency anemia, poor response to oral iron, and partial but incomplete response to intravenous iron therapy. Which is the genetic mutation associated with IRIDA?
Correct Answer: C
Rationale: The correct answer is C: TMPRSS6. Mutations in TMPRSS6 gene are associated with IRIDA. TMPRSS6 encodes matriptase-2, a key negative regulator of hepcidin, a hormone that controls iron absorption. Mutations in TMPRSS6 lead to impaired hepcidin regulation, resulting in reduced iron absorption and subsequent iron deficiency anemia. Choice A: TFR2 mutations are associated with hereditary hemochromatosis, not IRIDA. Choice B: H63D mutation in HFE gene is also associated with hereditary hemochromatosis, not IRIDA. Choice D: EPOR mutations are associated with congenital erythrocytosis, not IRIDA.
Question 5 of 5
The nurse is assessing an older client for any potential hematologic health problem. Which assessment finding is the most significant and would be reported to the primary health care provider?
Correct Answer: B
Rationale: The correct answer is B - Multiple petechiae and large bruises. This finding indicates potential bleeding issues or clotting disorders, which are serious hematologic health problems. Petechiae and bruises may suggest thrombocytopenia or other underlying conditions. Reporting this to the primary health care provider is crucial for further evaluation and management. A: Poor skin turgor on both forearms - Indicates dehydration, not necessarily a hematologic issue. C: Dry, flaky skin on arms and legs - Suggests a skin condition, not directly related to hematologic problems. D: Decreased body hair distribution - More indicative of endocrine or nutritional issues, not specific to hematologic health problems.