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: Step 1: Agreeing to wait until week 12 of therapy before resection allows chemotherapy to shrink the tumor, making surgery easier and potentially less extensive.
Step 2: Waiting until week 12 also helps assess the tumor's response to chemotherapy, guiding further treatment decisions.
Step 3: Choosing not to delay the resection until week 12 may risk more extensive surgery or complications due to the tumor's size.
Step 4: Opting for radiotherapy regardless of tumor resection status at week 12 is not advisable as unnecessary radiation exposure should be avoided unless absolutely necessary.
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Which of the following is a key feature of Factor XIII?
- A. Its half-life is about 10 days.
- B. It is an important activator of thrombin.
- C. Its levels are normal in newborns.
- D. It is part of the contact activation system.
Correct Answer: A
Rationale: The correct answer is A: Its half-life is about 10 days. Factor XIII has a long half-life due to its stable tetrameric structure, allowing it to circulate in the blood for about 10-14 days. This extended half-life is crucial for its role in stabilizing fibrin clots.
Explanation for why other choices are incorrect:
B: Factor XIII is not an activator of thrombin; rather, it crosslinks fibrin strands to strengthen clots.
C: Factor XIII levels are low in newborns and gradually increase during the first few months of life.
D: Factor XIII is not part of the contact activation system, which involves factors XII, XI, and prekallikrein.
A 50-year-old male who had frequent infections of his upper respiratory tract manifested with subcutaneous petechiae over his arms and legs. Investigations revealed low hemoglobin and a dry tap marrow. The most likely condition the patient is suffering from
- A. Iron deficiency anemia
- B. Megaloblastic anemia
- C. Hemolytic anemia
- D. Aplastic anemia
Correct Answer: D
Rationale: The correct answer is D: Aplastic anemia. Aplastic anemia is characterized by pancytopenia, which includes low hemoglobin levels leading to anemia. The dry tap marrow indicates bone marrow failure, which is a hallmark of aplastic anemia. The subcutaneous petechiae are due to thrombocytopenia, another feature of this condition. Iron deficiency anemia (A) typically presents with microcytic hypochromic red blood cells and is not associated with bone marrow failure. Megaloblastic anemia (B) is characterized by macrocytic red blood cells and is usually due to deficiencies in vitamin B12 or folate. Hemolytic anemia (C) involves the premature destruction of red blood cells and is not typically associated with bone marrow failure.
You are consulting on a 10-year-old male with severe persistent neutropenia, a history of recurrent infections, and warts. The rest of the peripheral blood count is normal. His mother also has neutropenia. Bone marrow examination shows a hypercellular marrow and retained myeloid cells with vacuolated cytoplasm. There are no abnormalities in the red cells or platelet precursors. Cytogenetics are 46XY. You start granulocyte colony stimulating factor therapy and the neutrophil count increases. A mutation in which of the following genes is most likely to have caused this familial inherited bone marrow failure syndrome?
- A. CXCR4
- B. ELANE
- C. GATA 2
- D. Mitochondrial DNA
Correct Answer: A
Rationale: The correct answer is A: CXCR4. In this case, the patient presents with severe neutropenia, recurrent infections, and warts, suggestive of WHIM syndrome, where CXCR4 mutations are often involved. CXCR4 plays a crucial role in immune cell trafficking and retention in the bone marrow. The hypercellular marrow and vacuolated myeloid cells are consistent with WHIM syndrome. The absence of abnormalities in red cells or platelet precursors rules out other syndromes. Mutations in ELANE are commonly associated with congenital neutropenia, not familial inherited bone marrow failure syndromes. GATA2 mutations are linked to familial myelodysplastic syndromes, not typically presenting with neutropenia and warts. Mitochondrial DNA mutations are more related to mitochondrial disorders, which usually manifest with multi-system involvement, not specific to bone marrow failure syndromes.
A 19-year-old freshman in college presents with 'lumps' on the right side of his neck and in the right axilla. He had a fever to 39 °C 1 day in the past week. On physical exam, there are firm anterior cervical and axillary nodes, all greater than 2 cm in diameter. A chest x-ray shows a large mediastinal mass. A biopsy of the axillary node reveals classic Hodgkin lymphoma. Which of the following symptoms revealed during the history is a B symptom?
- A. Fever to 39 °C
- B. 10% weight loss in past 6 months
- C. Fatigue
- D. Alcohol-induced pain
Correct Answer: B
Rationale: The correct answer is B. In Hodgkin lymphoma, B symptoms are systemic symptoms associated with poor prognosis. Weight loss of ≥10% in the past 6 months is a B symptom. This indicates a more aggressive disease. Fever (choice A) is a general symptom. Fatigue (choice C) is common but nonspecific. Alcohol-induced pain (choice D) is not a recognized B symptom in Hodgkin lymphoma. Weight loss is a key indicator of disease severity and is classified as a B symptom.
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.