Nokea
Several gene mutations have been associated with juvenile myelomonocytic leukemia (JMML), and they may or may not have prognostic implications. A gene expression–based classification system has been found to be an independent predictor of clinical outcome in these patients. What is the disease signature that predicts a poor outcome?
- A. Tyrosine kinase inhibitors
- B. Acute myeloid leukemia–like
- C. Chronic myeloid leukemia-like
- D. BRAF pathway abnormalities
Correct Answer: B
Rationale: The correct answer is B: Acute myeloid leukemia-like. This is because JMML shares similarities with acute myeloid leukemia in terms of aggressive progression and poor outcomes. Children with JMML who exhibit an acute myeloid leukemia-like gene expression signature have been shown to have a worse prognosis compared to those with other gene expression profiles. The other choices (A, C, D) are incorrect because tyrosine kinase inhibitors are not directly related to predicting clinical outcomes in JMML, chronic myeloid leukemia-like gene expression profile does not necessarily predict poor outcomes in JMML, and BRAF pathway abnormalities are not specifically associated with predicting poor outcomes in JMML.
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An 18-year old male patient with acute lymphoblastic leukemia recently started maintenance therapy and is complaining of increased hip pain. The pain increases during weight-bearing activity; however, it occasionally hurts at night as well. His CBCd is normal. Which of the following risk factors is most commonly associated with this process?
- A. Younger age at diagnosis
- B. Non-White race
- C. Low body-mass index
- D. Dexamethasone exposure
Correct Answer: D
Rationale: The correct answer is D: Dexamethasone exposure. Dexamethasone is a corticosteroid commonly used in the treatment of acute lymphoblastic leukemia. It can cause avascular necrosis of the hip due to its effect on bone metabolism, leading to increased hip pain during weight-bearing activities. This is a known side effect seen more commonly in patients exposed to dexamethasone.
Incorrect Choices:
A: Younger age at diagnosis - Age is not a known risk factor for avascular necrosis in this context.
B: Non-White race - Race is not a known risk factor for avascular necrosis in this context.
C: Low body-mass index - Body mass index is not a known risk factor for avascular necrosis in this context.
A nurse is caring for a patient who is being treated for leukemia in the hospital. The patient was able to maintain her nutritional status for the first few weeks following her diagnosis but is now exhibiting early signs and symptoms of malnutrition. In collaboration with the dietitian, the nurse should implement what intervention?
- A. Arrange for total parenteral nutrition (TPN).
- B. Facilitate placement of a percutaneous endoscopic gastrostomy (PEG) tube.
- C. Provide the patient with several small, soft-textured meals each day.
- D. Assign responsibility for the patient's nutrition to the patient's friends and family.
Correct Answer: C
Rationale: The correct answer is C: Provide the patient with several small, soft-textured meals each day. This intervention is appropriate because it focuses on improving the patient's nutritional intake through easily digestible meals, which can help address early signs of malnutrition. Small, soft-textured meals are easier for the patient to eat, especially if they are experiencing symptoms like mouth sores or difficulty swallowing. This approach also promotes regular intake of nutrients throughout the day, which can be more beneficial than relying solely on one large meal.
Incorrect answers:
A: Total parenteral nutrition (TPN) is typically reserved for patients who cannot tolerate oral or enteral nutrition. It is not the first-line intervention for early signs of malnutrition.
B: Percutaneous endoscopic gastrostomy (PEG) tube placement is usually considered for patients who are unable to eat orally in the long term. It is not indicated for early signs of malnutrition.
D: Assigning responsibility for the patient's nutrition to friends and
A 16-year-old female presents to the emergency room with a new complaint of chest pain. When performing a review of systems and physical examination, which of the following would substantially decrease your suspicion for a diagnosis of pulmonary embolism?
- A. Cough
- B. Fever
- C. Rib tenderness
- D. Shortness of breath
Correct Answer: C
Rationale: The correct answer is C: Rib tenderness. Rib tenderness is a symptom more commonly associated with musculoskeletal issues rather than pulmonary embolism. Pulmonary embolism typically presents with symptoms such as chest pain, shortness of breath, and sometimes cough. Fever is not a typical symptom of pulmonary embolism. Shortness of breath is a common symptom but not specific enough to substantially decrease suspicion for pulmonary embolism.
A patient with advanced leukemia is responding poorly to treatment. The nurse finds the patient tearful and trying to express his feelings, but he is clearly having difficulty. What is the nurse's most appropriate action?
- A. Tell him that you will give him privacy and leave the room.
- B. Offer to call pastoral care.
- C. Ask if he would like you to sit with him while he collects his thoughts.
- D. Tell him that you can understand how he's feeling.
Correct Answer: C
Rationale: The correct answer is C because it demonstrates empathy and support. By asking if the patient would like the nurse to sit with him, it shows willingness to provide comfort and assistance. This action allows the patient to feel supported while expressing his feelings.
Choice A is incorrect because leaving the patient alone may make him feel abandoned. Choice B is not the most immediate action needed in this situation. Choice D is incorrect because it assumes understanding without actively offering support.
You have been asked to see a 15-year-old girl who is being referred for evaluation of an ovarian mass. Her history is also significant for secondary amenorrhea, and physical examination shows signs of virilization. As you review her family history, what syndrome will you consider?
- A. Li-Fraumeni syndrome
- B. DICER-1 syndrome
- C. Turner syndrome
- D. Beckwith-Wiedemann syndrome
Correct Answer: B
Rationale: The correct answer is B: DICER-1 syndrome. This syndrome is associated with ovarian tumors, secondary amenorrhea, and signs of virilization due to androgen-secreting tumors. Li-Fraumeni syndrome (A) is characterized by multiple primary cancers but not specifically ovarian masses. Turner syndrome (C) presents with ovarian dysgenesis and primary amenorrhea. Beckwith-Wiedemann syndrome (D) is associated with overgrowth and abdominal wall defects, not ovarian masses. In this case, the presence of ovarian mass, secondary amenorrhea, and virilization point towards DICER-1 syndrome.