Nokea
An 8-year-old girl presents with National Cancer Institute (NCI) Standard Risk acute pre-B-cell acute lymphoblastic leukemia. Her family history is significant for her mother having been diagnosed with breast cancer at age 34 years and a maternal uncle who developed osteosarcoma as a teenager. What cytogenetic abnormality is most likely to be detected in this patient?
- A. t(1;19)
- B. CRLF2 rearrangement with a JAK2 mutation
- C. KMT24 rearrangement
- D. Hypodiploidy with a modal chromosome number of 34
Correct Answer: D
Rationale: The correct answer is D, Hypodiploidy with a modal chromosome number of 34. In pediatric pre-B-cell acute lymphoblastic leukemia, hypodiploidy with fewer than 44 chromosomes is associated with a poor prognosis. This cytogenetic abnormality is commonly seen in cases of NCI Standard Risk acute pre-B-cell ALL.
A: t(1;19) is typically associated with T-cell ALL, not pre-B-cell ALL.
B: CRLF2 rearrangement with a JAK2 mutation is more commonly seen in high-risk B-cell ALL.
C: KMT24 rearrangement is not a recognized cytogenetic abnormality in ALL.
In summary, the presence of hypodiploidy with a modal chromosome number of 34 is the most likely cytogenetic abnormality in this patient based on her clinical presentation and family history.
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A 16-year-old female patient with severe factor XI deficiency presents with acute appendicitis and requires urgent surgery. You are called by the surgeon, who wants to know what, if any, blood products or treatments are required to reduce the risk of perioperative bleeding. The patient weighs 62 kg. What should you tell him to administer?
- A. Cryoprecipitate (five units), which will likely raise her factor XI level to 20%
- B. Factor XI concentrate (20 units/kg), which will raise her factor XI level to 20%
- C. Fresh frozen plasma (20 mL/kg), which will raise her factor XI level to 20%
- D. Prothrombin complex concentrate (40 units/kg), which will raise her factor XI level to 20%
Correct Answer: C
Rationale: The correct answer is C: Fresh frozen plasma (20 mL/kg), which will raise her factor XI level to 20%. Fresh frozen plasma contains various clotting factors, including factor XI. In a patient with severe factor XI deficiency, administering fresh frozen plasma can increase factor XI levels and help reduce the risk of perioperative bleeding. The dose of 20 mL/kg is appropriate for this patient's weight of 62 kg. Cryoprecipitate (choice A) may contain factor XI but is not the optimal choice for raising factor XI levels specifically. Factor XI concentrate (choice B) would be ideal but is not commonly available, making fresh frozen plasma a more practical option. Prothrombin complex concentrate (choice D) primarily contains factors II, VII, IX, and X, but not factor XI, so it would not effectively raise factor XI levels in this patient.
A 9-year-old boy presents to the emergency department with emesis and headache of 3 weeks' duration. MRI reveals a large heterogeneous mass in the cerebellum. He undergoes a resection, and the pathology is most consistent with a classic medulloblastoma. Which of the following findings would classify the patient as a high-risk medulloblastoma?
- A. Elevated serum and CSF AFP and beta-HCG tumor markers
- B. WNT subgrouping on molecular classification
- C. A presurgical spine MRI that reveals bulky tumor in the spine
- D. A postoperative brain MRI with no signs of residual tumor
Correct Answer: C
Rationale: The correct answer is C. A presurgical spine MRI revealing bulky tumor in the spine classifies the patient as high-risk due to leptomeningeal spread. This indicates dissemination of the tumor cells beyond the primary site, leading to a poorer prognosis. Choice A is incorrect as AFP and beta-HCG markers are associated with germ cell tumors, not medulloblastomas. Choice B is incorrect as WNT subgrouping is associated with better outcomes. Choice D is incorrect because the absence of residual tumor postoperatively does not necessarily indicate high-risk disease.
A client is having a radioisotopic imaging scan. What action by the nurse is most important?
- A. Assess the client for shellfish allergies.
- B. Place the client on radiation precautions.
- C. Sedate the client before the scan.
- D. Teach the client about the procedure.
Correct Answer: D
Rationale: The correct answer is D because educating the client about the procedure is crucial for informed consent, reducing anxiety, and promoting cooperation during the scan. This empowers the client to understand what to expect and how to prepare. Assessing for shellfish allergies (A) is not directly related to the scan. Placing the client on radiation precautions (B) is unnecessary as the exposure is minimal, and sedating the client (C) should only be considered if medically indicated, not as a routine practice.
A 50-year-old male who had frequent infections of his upper respiratory tract manifested with subcutaneous petechiae over his arms and legs. Investigations revealed low hemoglobin and a dry tap marrow. The most likely condition the patient is suffering from
- A. Iron deficiency anemia
- B. Megaloblastic anemia
- C. Hemolytic anemia
- D. Aplastic anemia
Correct Answer: D
Rationale: The correct answer is D: Aplastic anemia. This condition is characterized by pancytopenia, which includes low hemoglobin (anemia), low platelets (manifested as petechiae), and low white blood cells (frequent infections). Dry tap marrow is a classic finding in aplastic anemia due to bone marrow failure. Iron deficiency anemia (choice A) typically presents with microcytic hypochromic anemia without pancytopenia. Megaloblastic anemia (choice B) is characterized by macrocytic anemia with hypersegmented neutrophils and is usually caused by deficiencies in vitamin B12 or folate. Hemolytic anemia (choice C) presents with anemia due to increased destruction of red blood cells, which does not explain the low platelets and white blood cells seen in this patient.
A patient has an altered level of T and B cells. The nurse realizes that these cells are members of which cell type?
- A. Platelets
- B. Eosinophils
- C. Lymphocytes
- D. Red blood cells
Correct Answer: C
Rationale: The correct answer is C: Lymphocytes. T and B cells are types of lymphocytes, a crucial component of the immune system responsible for fighting infections. T cells regulate immune responses, while B cells produce antibodies. Platelets (A), eosinophils (B), and red blood cells (D) are not involved in immune responses and do not possess the functions of T and B cells. Platelets are involved in blood clotting, eosinophils are a type of white blood cell involved in allergic reactions, and red blood cells are responsible for oxygen transport.