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 (fewer than 44 chromosomes) is associated with poor prognosis. A modal chromosome number of 34 is specifically linked to high-risk disease.
A: t(1;19) is characteristic of pre-B-cell acute lymphoblastic leukemia but is associated with intermediate prognosis, not standard risk.
B: CRLF2 rearrangement with a JAK2 mutation is seen in a subset of high-risk acute lymphoblastic leukemia cases, not standard risk.
C: KMT24 rearrangement is not a known cytogenetic abnormality in acute lymphoblastic leukemia.
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A patient is prescribed a transfusion of washed packed red blood cells. What should the nurse realize as being the rationale for the using this type of blood?
- A. Reduces the risk of hypothermia
- B. Cleans the blood cells of impurities
- C. Reduces the risk of a febrile reaction
- D. Removes potential harmful particles from the blood
Correct Answer: C
Rationale: The correct answer is C because washing packed red blood cells reduces the risk of a febrile reaction by removing white blood cells, plasma proteins, and other substances that could lead to an immune response. Choice A is incorrect as hypothermia is not a primary concern with washed packed red blood cells. Choice B is incorrect as the process does not cleanse blood cells of impurities. Choice D is incorrect as the main purpose of washing blood cells is to reduce the risk of adverse reactions, not to remove harmful particles.
You are caring for a patient with a large localized Ewing sarcoma of the soft tissues of the arm. The surgeon believes that the tumor can be resected without amputation but asks whether you can give some chemotherapy to shrink the tumor before surgery. Which of the following would you tell the surgeon?
- A. If the tumor can be resected without amputation, then the best time to do the resection is before any chemotherapy to improve the prognosis.
- B. You agree with waiting to do the resection until week 12 of therapy and will begin chemotherapy; you recognize that radiotherapy will not be necessary if the tumor is completely resected at week 12 of therapy.
- C. You agree with waiting to do the resection until week 12 of therapy and will begin chemotherapy; you recognize that radiotherapy will be necessary even if the tumor is completely resected at week 12 of therapy.
- D. If the tumor can be resected without amputation, then the best time to do the resection is before any chemotherapy; you recognize that this is the only way to avoid radiotherapy.
Correct Answer: B
Rationale: The correct answer is B because starting chemotherapy before resection at week 12 can help shrink the tumor, making it more manageable for surgery. A is incorrect because preoperative chemotherapy can improve outcomes. C is incorrect because radiotherapy may not be necessary if the tumor is completely resected. D is incorrect because radiotherapy may still be necessary even if the tumor is resected before chemotherapy.
A nurse is caring for a client who is about to begin therapy with recombinant factor IX to treat hemophilia B. The client asks the nurse about the risk of disease transmission with recombinant factor IX as compared with plasma derived factor IX. The nurse should explain that the recombinant factor IX practically eliminates the risk for which of the following?
- A. HIV
- B. Cytomegalovirus
- C. Creutzfeldt-Jakob disease
- D. Anaphylaxis
Correct Answer: C
Rationale: Rationale: The correct answer is C: Creutzfeldt-Jakob disease (CJD). Recombinant factor IX is produced synthetically in a laboratory, eliminating the risk of transmitting prion diseases like CJD. Plasma-derived products, on the other hand, carry a theoretical risk of transmitting CJD due to potential contamination. HIV and cytomegalovirus can be transmitted through blood products, but both plasma-derived and recombinant factor IX are rigorously tested for these viruses. Anaphylaxis is a potential risk with any factor IX product, regardless of the source. Therefore, the nurse should explain to the client that recombinant factor IX practically eliminates the risk of CJD transmission compared to plasma-derived factor IX.
RBCs break into fragments, they are referred to as schistocytes. These type of cells are seen in
- A. Sickle cell trait
- B. Thalassemia
- C. Microangiopathic hemolytic anemia
- D. Idiopathic thrombocytopenic purpura
Correct Answer: C
Rationale: The correct answer is C: Microangiopathic hemolytic anemia. Schistocytes are fragmented red blood cells seen in conditions where RBCs are physically sheared or damaged as they pass through small blood vessels. In microangiopathic hemolytic anemia, abnormal or damaged blood vessels cause mechanical destruction of RBCs, leading to the formation of schistocytes. Sickle cell trait (A) and Thalassemia (B) are genetic disorders that do not typically cause schistocytes. Idiopathic thrombocytopenic purpura (D) is a condition characterized by low platelet count, not associated with schistocytes.
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?
- A. Skin fibroblasts to evaluate the RUNXI gene
- B. Skin fibroblasts to evaluate the ETV6 gene
- C. Buccal swab to evaluate the RUNXI gene
- D. Buccal swab to evaluate the ETV6 gene
Correct Answer: B
Rationale: The correct answer is B: Skin fibroblasts to evaluate the ETV6 gene. In individuals with familial thrombocytopenia and a history of malignancy, ETV6 gene mutations are often implicated. Sending skin fibroblasts for analysis allows for genetic testing to identify any ETV6 gene mutations that may be present. Skin fibroblasts are commonly used for genetic testing as they provide a stable and reliable source of DNA.
Choice A (Skin fibroblasts to evaluate the RUNXI gene) is incorrect because RUNXI gene mutations are not typically associated with familial thrombocytopenia and cancer predisposition syndromes. Choice C (Buccal swab to evaluate the RUNXI gene) is also incorrect for the same reason. Choice D (Buccal swab to evaluate the ETV6 gene) is incorrect because buccal swabs may not provide enough genetic material for comprehensive testing of the ETV6 gene, which is