You receive a phone call that a 3-year-old patient on long-term warfarin therapy for congenital heart disease has an international normalized ratio (INR) of 5.8. On further history, you learn the patient and several family members have had recent gastrointestinal illnesses, but the patient is recovering. His mother reports he is not experiencing bleeding symptoms. Which of the following interventions would be most reasonable in this clinical scenario?
- A. Hold 1 to 2 doses of warfarin and recheck INR
- B. Administer oral vitamin K therapy
- C. Administer fresh frozen plasma (FFP)
- D. Administer recombinant factor VIIa
Correct Answer: A
Rationale: The correct answer is A: Hold 1 to 2 doses of warfarin and recheck INR. In this scenario, the patient's elevated INR of 5.8 indicates an increased risk of bleeding due to excessive anticoagulation. Since the patient is not experiencing bleeding symptoms and is recovering from gastrointestinal illness, temporarily holding 1 to 2 doses of warfarin is the most reasonable intervention to prevent bleeding complications while allowing the INR to normalize. Rechecking the INR after holding the doses will help assess the patient's response to the intervention. Choices B, C, and D are incorrect because administering oral vitamin K therapy, FFP, or recombinant factor VIIa are more aggressive interventions that are not warranted in this case where the patient is asymptomatic and recovering from a transient illness.
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An older adult patient is receiving a transfusion of packed red blood cells after being injured in a car accident. On assessment, the nurse notes a new finding of bounding pulse, crackles, and increasing dyspnea. What should the nurse do first, after stopping the transfusion?
- A. Assess vital signs.
- B. Raise the head of the bed.
- C. Encourage the patient to deep breathe and cough.
- D. Administer pm diphenhydramine (Benadryl) as ordered.
Correct Answer: B
Rationale: The correct answer is B: Raise the head of the bed. This action is crucial in managing the potential complication of fluid overload in the patient receiving a blood transfusion. Elevating the head of the bed helps reduce venous return to the heart, decreasing preload and cardiac workload. This can alleviate symptoms like dyspnea and crackles associated with fluid overload. Assessing vital signs (choice A) is important but should follow positioning the patient appropriately. Encouraging deep breathing and coughing (choice C) may exacerbate the patient's respiratory distress. Administering diphenhydramine (choice D) is not indicated for the symptoms described.
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.
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 receive a phone call that a 3-year-old patient on long-term warfarin therapy for congenital heart disease has an international normalized ratio (INR) of 5.8. On further history, you learn the patient and several family members have had recent gastrointestinal illnesses, but the patient is recovering. His mother reports he is not experiencing bleeding symptoms. Which of the following interventions would be most reasonable in this clinical scenario?
- A. Hold 1 to 2 doses of warfarin and recheck INR
- B. Administer oral vitamin K therapy
- C. Administer fresh frozen plasma (FFP)
- D. Administer recombinant factor VIIa
Correct Answer: A
Rationale: The correct answer is A: Hold 1 to 2 doses of warfarin and recheck INR. In this scenario, the high INR indicates an increased risk of bleeding due to excess anticoagulation. However, since the patient is asymptomatic and the high INR is likely due to transient factors (gastrointestinal illness), the most appropriate initial step is to temporarily hold warfarin to allow the INR to normalize. Rechecking the INR after holding the doses will guide further management.
Choice B (Administer oral vitamin K therapy) is incorrect because the patient is not experiencing bleeding symptoms and oral vitamin K should be reserved for patients with active bleeding or high INR with bleeding symptoms. Choice C (Administer fresh frozen plasma) is inappropriate as the patient is not actively bleeding and FFP is typically reserved for acute bleeding situations. Choice D (Administer recombinant factor VIIa) is also incorrect as it is reserved for severe bleeding in patients on war
Presence of an --jaundice --splenomegaly with increase MCH is seen in:
- A. liver cirrhosis
- B. th.major
- C. PNH
- D. herditary spherocytosis
Correct Answer: D
Rationale: The correct answer is D, hereditary spherocytosis. In this condition, the presence of jaundice and splenomegaly is due to the destruction of red blood cells by the spleen, leading to increased MCH. In liver cirrhosis (choice A), jaundice is due to impaired liver function. Thalassemia major (choice B) presents with severe anemia, not jaundice. Paroxysmal nocturnal hemoglobinuria (choice C) is characterized by hemolytic anemia and not typically associated with splenomegaly and jaundice. Therefore, the presence of jaundice, splenomegaly, and increased MCH points towards hereditary spherocytosis.