Nokea
Which of the following statements about myeloablative, myeloablative but reduced toxicity, reduced intensity, and non-myeloablative approaches is not correct?
- A. Myeloablative approaches are needed for high-risk malignancies to maximize depth of remission and decrease the likelihood of relapse.
- B. Reduced intensity regimens can be successfully used for most nonmalignant disorders to minimize risk of late effects.
- C. Reduced intensity regimens can markedly decrease the risk of transplant-related mortality in patients who have underlying significant comorbidities but at the cost of more relapse and possibly more graft-versus-host disease.
- D. Non-myeloablative regimens are used for the very highest risk patients to minimize toxicity and for certain diseases such as aplastic anemia.
Correct Answer: B
Rationale: The correct answer is B because reduced intensity regimens are used to minimize toxicity and late effects, not for most nonmalignant disorders. Myeloablative approaches are needed for high-risk malignancies to maximize remission depth (A), reduced intensity regimens can increase transplant-related mortality in high-risk patients (C), and non-myeloablative regimens are used for the highest risk patients and certain diseases like aplastic anemia (D).
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A patient with pancytopenia has a bone marrow aspiration from the left posterior iliac crest. Which action would be important for the nurse to take after the procedure?
- A. Elevate the head of the bed to 45 degrees.
- B. Apply a sterile 2-inch gauze dressing to the site.
- C. Use a half-inch sterile gauze to pack the wound.
- D. Have the patient lie on the left side for 1 hour.
Correct Answer: D
Rationale: The correct answer is D: Have the patient lie on the left side for 1 hour. This position helps prevent bleeding by applying pressure to the site. Elevating the head of the bed (A) is not necessary for this procedure. Applying a 2-inch gauze dressing (B) may disrupt the site and increase the risk of bleeding. Using half-inch sterile gauze to pack the wound (C) is not recommended for bone marrow aspiration sites as it can lead to infection.
A 4-year-old male child presents to the emergency department with his fourth invasive Staph infection. CBC consistently identifies moderate neutropenia. Sophisticated lab testing identifies lack of Toll-like receptor responses. The patient undergoes whole exome sequencing and is found to have pathogenic variants in IRAK4. What does 'IRAK4' stand for?
- A. Interferon gamma receptor-associated kinase 4
- B. Inducible RAS activating kinase 4
- C. Interleukin-1 receptor-associated kinase 4
- D. Immune response activating kinase 4
Correct Answer: C
Rationale: The correct answer is C: Interleukin-1 receptor-associated kinase 4 (IRAK4).
1. IRAK4 is involved in the immune response pathway triggered by interleukin-1 receptor signaling.
2. Lack of Toll-like receptor responses in the patient aligns with the role of IRAK4 in the interleukin-1 receptor pathway.
3. Pathogenic variants in IRAK4 can lead to immunodeficiency, explaining recurrent Staph infections.
4. Choices A, B, and D do not accurately reflect the known function of IRAK4 and its association with interleukin-1 receptor signaling.
A nurse is caring for four clients. After reviewing today's laboratory results, which client should the nurse see first?
- A. Client with an international normalized ratio of 2.8
- B. Client with a platelet count of 128000/mm³
- C. Client with a prothrombin time (PT) of 28 seconds
- D. Client with a red blood cell count of 5.1 million/L
Correct Answer: C
Rationale: The correct answer is C. The nurse should see the client with a prothrombin time (PT) of 28 seconds first because PT measures the time it takes for blood to clot. A PT of 28 seconds is prolonged, indicating potential bleeding risk. This requires immediate attention to prevent complications.
Choice A: A client with an international normalized ratio of 2.8 may indicate anticoagulant therapy but is not as urgent as a prolonged PT.
Choice B: A platelet count of 128,000/mm³ is low but doesn't necessarily require immediate intervention compared to a prolonged PT.
Choice D: A red blood cell count of 5.1 million/L is within normal range and does not indicate an urgent issue related to clotting or bleeding.
A 3-month-old female presents to the emergency room with vomiting and abdominal distension. She has a left-side abdominal mass, and an abdominal ultrasound confirms an 8-cm mass arising from the left kidney. Liver lesions are also noted. Nephrectomy is performed and reveals a histologic diagnosis of malignant rhabdoid tumor of the kidney (MRTK). Which of the following is not a true statement about the management of this patient?
- A. Most patients with rhabdoid tumor of the kidney present in infancy.
- B. Most patients with rhabdoid tumor of the kidney present with metastatic (stage III or IV) disease.
- C. She has an excellent prognosis with surgery, chemotherapy, and radiation.
- D. Germline testing for SMARCB1/INI1 mutation on chromosome 22 is recommended, with brain MRI every 3 months until she is 5 years old, if testing is germline positive for SMARCB1/INI1.
Correct Answer: C
Rationale: The correct answer is C: She has an excellent prognosis with surgery, chemotherapy, and radiation. This statement is not true because malignant rhabdoid tumor of the kidney (MRTK) has a poor prognosis, even with aggressive treatment. Here's a breakdown:
1. MRTK is an aggressive and rare tumor that often presents in infancy, supporting statement A.
2. Most patients with MRTK present with metastatic disease, indicating poor prognosis, aligning with statement B.
3. Germline testing for SMARCB1/INI1 mutation is essential due to the genetic predisposition associated with MRTK, supporting statement D.
In summary, statement C is incorrect as MRTK typically has a challenging clinical course despite comprehensive treatment approaches.
You have a new 7-year-old female patient with a WBC count of 6,000/mm3, hemoglobin of 7.2 g/dL, and platelet count of 30,000/mm3. A bone marrow aspirate reveals 14% blasts with a monocytic morphologic appearance that are surface marker positive for CD33. You receive a call from the fluorescence in situ hybridization (FISH) lab that the bone marrow is positive for KMT2A rearrangement in 68% of cells. Your staff asks whether this represents a diagnosis of acute leukemia in the current classification scheme for this type of hematologic malignancy. What would you say?
- A. No, because for a diagnosis of acute leukemia you must have 30% or more blasts in the marrow.
- B. No, because for a diagnosis of acute leukemia you must have 20% or more blasts in the marrow.
- C. No, because the cytogenetics do not include +21, monosomy 7, or trisomy 8.
- D. Yes, because the FISH is positive for KMT2A rearrangement.
Correct Answer: D
Rationale: The correct answer is D: Yes, because the FISH is positive for KMT2A rearrangement. The presence of KMT2A rearrangement in 68% of cells indicates a specific genetic abnormality associated with acute leukemia. This abnormality is a known marker for acute myeloid leukemia (AML) with recurrent genetic abnormalities. The percentage of blasts in the bone marrow (14%) may not meet the traditional criteria for a diagnosis of acute leukemia based on blast percentage alone, but the presence of KMT2A rearrangement overrides this requirement in this case. Choices A and B are incorrect because blast percentage alone is not the sole determinant of a diagnosis of acute leukemia when specific genetic abnormalities are present. Choice C is incorrect because while the absence of specific cytogenetic abnormalities may be relevant for some cases, the presence of KMT2A rearrangement is sufficient to support a diagnosis of acute leukemia in this context.