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 in managing this patient with a Wilms tumor. Nephrectomy is the primary treatment for Wilms tumor to remove the primary tumor and obtain tissue for histologic diagnosis. Lymph node sampling helps determine if there is any spread of the tumor. Chemotherapy based on histology and stage is tailored to the specific characteristics of the tumor and extent of disease. Choice A (Core biopsy of the renal mass followed by three drug chemotherapy) is incorrect because core biopsy may not provide enough tissue for a definitive diagnosis, and treatment should be guided by surgical resection. Choice C (Fine-needle aspiration followed by chemotherapy) is incorrect because fine-needle aspiration may not provide adequate tissue for diagnosis, and surgical resection is needed for definitive management. Choice D (Neoadjuvant chemotherapy followed by nephrectomy) is incorrect because nephrectomy is
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, about 50% of cases result from inversion mutations in the F8 gene. In this case, since there is no family history of hemophilia or other bleeding disorders, the most likely scenario is a de novo genetic mutation, such as an inversion mutation. This type of mutation can occur spontaneously and is not inherited from parents. Therefore, genotyping the Factor VIII gene in this newborn male with severe hemophilia A is likely to reveal an inversion mutation as the underlying genetic cause. Choice A is incorrect because the absence of a family history does not rule out the presence of a de novo mutation. Choice B (missense mutation) and Choice D (nonsense mutation) are less likely in hemophilia A compared to inversion mutations. Missense and nonsense mutations are more commonly associated with other genetic conditions or types of hemophilia.
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's presentation with thrombosis of the right upper extremity, anatomical compression/narrowing of the right subclavian vein, and being a high school baseball pitcher suggests Paget-Schroetter syndrome (effort thrombosis). The first rib can compress the subclavian vein in people with certain anatomical variations, leading to thrombosis. Resection of the right first rib (first rib resection) is the definitive treatment for this condition, as it removes the mechanical compression, reducing the risk of recurrent thrombosis. A: Systemic thrombolysis involves using medications to dissolve blood clots throughout the body and is not the first-line treatment for Paget-Schroetter syndrome. B: Catheter-directed thrombolysis is not indicated for anatomical compression/narrowing as seen in this patient. C: Extended anticoagulation with LMWH does not address the underlying mechanical
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: Correct Answer: C (TMPRSS6) Rationale: 1. TMPRSS6 gene encodes matriptase-2, a negative regulator of hepcidin, which controls iron absorption. 2. Mutations in TMPRSS6 lead to elevated hepcidin levels, causing iron-refractory iron deficiency anemia. 3. TFR2, H63D, and EPOR are not directly involved in hepcidin regulation or iron absorption. Summary: A (TFR2), B (H63D), and D (EPOR) are not associated with the pathophysiology of IRIDA, which is primarily linked to mutations in the TMPRSS6 gene affecting hepcidin regulation and iron absorption.
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 is indicative of potential hematologic health problems such as thrombocytopenia or clotting disorders, which can be serious and require immediate medical attention. Petechiae are small, pinpoint hemorrhages under the skin, and large bruises suggest abnormal bleeding. Poor skin turgor (choice A) is more indicative of dehydration. Dry, flaky skin (choice C) may be related to skin conditions or inadequate hydration. Decreased body hair distribution (choice D) could be related to hormonal imbalances or aging, but it is not as concerning as signs of abnormal bleeding.