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. In JMML, a disease signature resembling acute myeloid leukemia (AML) has been associated with a poor outcome. This signature includes features such as increased blasts, abnormal karyotypes, and mutations in genes like NRAS and KRAS. AML-like JMML cases often have aggressive disease progression and poorer response to treatment. Tyrosine kinase inhibitors (choice A) are not typically used in JMML treatment. Chronic myeloid leukemia-like (choice C) is not associated with a poor outcome in JMML. BRAF pathway abnormalities (choice D) may be present in some cases of JMML but are not the primary disease signature predicting poor outcomes.
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A 5-year-old boy is evaluated for apparent ongoing hemolysis. His hemoglobin is 9.5 g/dL, with 8% reticulocytes and MCV 87 fL. Platelets and leukocytes are normal. His direct antiglobulin test (DAT) is negative. No cold agglutinin is detectable. His family history is negative for blood disorders. Peripheral smear reveals basophilic stippling in 10% of the red blood cells. Given these findings, which of the following blood disorders is most likely?
- A. Rh-null disease
- B. Hereditary pyropoikilocytosis
- C. Glucose phosphate isomerase deficiency
- D. Unstable hemoglobin
Correct Answer: E
Rationale: I'm sorry, but it seems like there might be a mistake in the question as there is no option "E" provided. Could you please provide the correct answer choice so that I can give you a step-by-step rationale for why that answer is correct?
According to the FAB classification, the L2 stage is characterized by:
- A. Large heterogenous blasts with prominent nucleoli
- B. Small homogenous blasts with scanty nucleoli and higher nucleus to cytoplasm ratio
- C. Large homogenous blasts with scanty nucleoli and higher nucleus to cytoplasm ratio
- D. Large blasts with basophilic vacuolated cytoplasm
Correct Answer: C
Rationale: The correct answer is C because according to the FAB classification, the L2 stage is characterized by large homogenous blasts with scanty nucleoli and a higher nucleus to cytoplasm ratio. This stage represents acute lymphoblastic leukemia with a high risk of relapse. Choice A is incorrect as it describes the L1 stage. Choice B is incorrect as it describes the L3 stage. Choice D is incorrect as it does not accurately describe the characteristics of the L2 stage.
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.
You are seeing a 12-year-old female who presented to the emergency department with the sudden onset of severe abdominal pain. Imaging that was obtained to rule out appendicitis revealed a mass adjacent to the bladder. The mass was surgically resected, and pathology demonstrated a paraganglioma. Which of the studies below would be most useful to determine disease stage for this patient?
- A. Bone Scan
- B. Lumbar puncture for cerebrospinal fluid cytology
- C. Bone marrow aspirate and biopsy
- D. Ga 68-DOTATATE PET/CT
Correct Answer: D
Rationale: The correct answer is D: Ga 68-DOTATATE PET/CT. This imaging study is the most useful for determining the disease stage in a patient with paraganglioma. Paragangliomas are neuroendocrine tumors that express somatostatin receptors, which can be detected using Ga 68-DOTATATE PET/CT. This imaging modality helps to localize primary and metastatic lesions, as well as assess disease extent and stage.
Now let's analyze the other options:
A: Bone Scan - Not useful for determining disease stage in paraganglioma.
B: Lumbar puncture for cerebrospinal fluid cytology - Not indicated for staging paraganglioma.
C: Bone marrow aspirate and biopsy - Not specific for staging paraganglioma.
In summary, Ga 68-DOTATATE PET/CT is the most appropriate study for determining disease stage in a patient with paraganglioma due to its
The health care provider tells the nurse that a client is to be started on a platelet inhibitor. About what drug does the nurse plan to teach the client?
- A. Clopidogrel (Plavix)
- B. Enoxaparin (Lovenox)
- C. Reteplase (Retavase)
- D. Warfarin (Coumadin)
Correct Answer: A
Rationale: The correct answer is A: Clopidogrel (Plavix). Clopidogrel is a platelet inhibitor that helps prevent blood clots by inhibiting platelet aggregation. It is commonly used to reduce the risk of heart attack or stroke. Enoxaparin (Lovenox) is a low molecular weight heparin that works by preventing blood clots by inhibiting clotting factors. Reteplase (Retavase) is a thrombolytic agent used to dissolve blood clots in emergency situations. Warfarin (Coumadin) is an anticoagulant that works by inhibiting vitamin K-dependent clotting factors. Clopidogrel is the most appropriate choice for a platelet inhibitor in this scenario due to its mechanism of action and indication for preventing blood clots.