Nokea
A 5-year-old boy presents with a 3-week history of his right eye “not moving to the right side.†The remainder of his neurologic exam is normal with the exception of a weak gag. MRI of the brain reveals a diffusely infiltrative mass in the pons. If this patient were to undergo a stereotactic biopsy, what would be the most likely molecular finding?
- A. BRAFKIAA1549 fusion
- B. Trisomy 21
- C. H3 K27M mutation
- D. RELA fusion
Correct Answer: C
Rationale: The correct answer is C: H3 K27M mutation. In children with diffuse midline gliomas, the most common molecular finding is the H3 K27M mutation. This mutation is associated with a worse prognosis and is commonly found in tumors located in the pons, which is consistent with the MRI findings in this case.
Choice A (BRAFKIAA1549 fusion) is incorrect as this fusion is typically associated with pilocytic astrocytomas, which are low-grade tumors and not typically found in the pons.
Choice B (Trisomy 21) is incorrect as it is a chromosomal abnormality associated with Down syndrome, not a molecular finding in brain tumors.
Choice D (RELA fusion) is incorrect as this fusion is typically found in ependymomas, which are not typically located in the pons.
You may also like to solve these questions
A nurse is monitoring a client following ferrous sulfate administration. The nurse should monitor the client for which of the following adverse effects?
- A. Phlebitis
- B. Dark
- C. orange-colored stools
- D. Constipation
Correct Answer: D
Rationale: The correct answer is D: Constipation. Ferrous sulfate is an iron supplement commonly associated with gastrointestinal side effects, including constipation. Iron can cause constipation by slowing down bowel movements. Phlebitis (A) is inflammation of a vein and is not a common adverse effect of ferrous sulfate. Dark (B) or orange-colored stools (C) are expected side effects of iron supplements due to the change in color caused by the iron, but they are not harmful. Constipation (D) is the most concerning adverse effect to monitor for as it can lead to discomfort and potential complications if left untreated.
A 13-year-old girl presents with acute myeloid leukemia (AML) and a WBC count of 120,000/mm3. Cytogenetics reveals a normal karyotype, and fluorescence in situ hybridization (FISH) tests for inv(16), t(8;21), t(15;17); 11q23 abnormalities; monosomy 7; and 5q deletion are negative. Molecular testing is negative for mutations in FLT3, NPM1, and CEBPA. She is treated with 10 days of daunorubicin, AraC, and gemtuzumab for induction therapy. On day 30, she recovers counts, and a bone marrow aspiration shows 2.2% leukemic blasts by flow cytometry. She receives a second course of treatment with daunorubicin and AraC, and her marrow is now in morphologic remission and is MRD-negative by flow cytometry. She has no HLA-matched siblings, but an unrelated donor search reveals a large number of potential matches. Which course of treatment is most likely to result in the best outcome?
- A. Give two more courses of intensification chemotherapy.
- B. Perform an autologous hematopoietic stem cell transplant (HSCT).
- C. Give one more course of intensification chemotherapy and then perform a matched unrelated donor HSCT.
- D. Give one more course of intensification chemotherapy and then 1 year of maintenance chemotherapy.
Correct Answer: C
Rationale: The correct answer is C: Give one more course of intensification chemotherapy and then perform a matched unrelated donor HSCT.
Rationale:
1. The patient achieved morphologic remission and MRD-negative status after the second course of chemotherapy, indicating good response.
2. Given the high-risk AML with negative cytogenetics and molecular markers, HSCT from a matched unrelated donor offers the best chance for long-term remission.
3. HSCT provides a curative option by replacing the patient's diseased bone marrow with healthy donor cells, reducing the risk of relapse.
4. The presence of a large number of potential matched unrelated donors increases the likelihood of finding a suitable donor for the transplant.
Summary:
- Option A: Giving more courses of intensification chemotherapy may not address the high-risk nature of the disease and may not provide a curative outcome.
- Option B: Autologous HSCT uses the patient's own stem cells, which may carry the risk of relapse due
A 4-year-old girl with a history of recurrent epistaxis and easy bruising is referred to you for evaluation. She is found to have a prolonged PTT and a factor VIII level that is less than 1%. Both parents have a history of excessive bleeding. She is admitted with a severe episode of epistaxis, and your colleague orders 40 IU/kg of recombinant factor VIII. Her epistaxis resolves initially but within an hour starts again at the same severity as before. What is the best next step?
- A. Infuse a von Willebrand factor concentrate.
- B. Give another dose of recombinant factor VIII concentrate.
- C. Call otorhinolaryngology to pack her nose.
- D. Check for a factor VIII inhibitor.
Correct Answer: A
Rationale: The correct answer is A: Infuse a von Willebrand factor concentrate. In this scenario, the 4-year-old girl has a history of recurrent epistaxis and easy bruising, indicative of a bleeding disorder. The prolonged PTT and factor VIII level less than 1% suggest a deficiency in von Willebrand factor (VWF) or factor VIII. When the initial dose of recombinant factor VIII did not completely resolve the epistaxis, it indicates a possible deficiency in VWF activity. Therefore, the best next step is to infuse a von Willebrand factor concentrate to address the VWF deficiency, which should help control the bleeding.
Summary:
- Option B (Give another dose of recombinant factor VIII concentrate) is not the best choice because the initial dose did not fully resolve the bleeding, indicating a different factor may be deficient.
- Option C (Call otorhinolaryngology to pack her nose) addresses the symptom but does not
The nurse examines the lymph nodes of a patient during a physical assessment. Which assessment finding would be of most concern to the nurse?
- A. A 2-cm nontender supraclavicular node
- B. A 1-cm mobile and nontender axillary node
- C. An inability to palpate any superficial lymph nodes
- D. Firm inguinal nodes in a patient with an infected foot
Correct Answer: A
Rationale: The correct answer is A. A 2-cm nontender supraclavicular node is of most concern as supraclavicular nodes are not normally palpable, and their enlargement can indicate underlying malignancy, such as metastatic cancer.
B: A 1-cm mobile and nontender axillary node is within normal limits.
C: Inability to palpate superficial lymph nodes may be due to factors like obesity or normal variation.
D: Firm inguinal nodes in a patient with an infected foot are expected as a local response to infection.
When should autologous hematopoietic stem cell transplantation be used, and what are the common cancers it is used for?
- A. It should be used when high dose therapy is needed to maximize response. Most common cancers it is used for include lymphoma, late relapse of acute lymphoblastic leukemia, neuroblastoma, and Ewing sarcoma.
- B. It should be used any time this approach can provide a meaningful survival benefit over chemotherapy. Most common cancers it is used for include neuroblastoma, responsive brain tumors in young children to avoid/minimize early radiation therapy, and relapsed lymphoma.
- C. It should be used for tumors in which a graft-versus-tumor effect does not occur. Most common cancers include neuroblastoma, lymphoma, selected brain tumors, rhabdomyosarcoma, and Ewing sarcoma with lung metastases.
- D. It should be used to avoid extensive treatment with chemotherapy and to shorten treatment. Most common cancers include neuroblastoma, relapsed Wilms' tumor, and selected brain tumors.
Correct Answer: B
Rationale: Correct Answer: B
Rationale:
1. Autologous hematopoietic stem cell transplantation should be used when it can provide a meaningful survival benefit over chemotherapy.
2. The most common cancers it is used for include neuroblastoma, responsive brain tumors in young children to avoid/minimize early radiation therapy, and relapsed lymphoma.
3. This choice aligns with the current guidelines and evidence-based practice in utilizing autologous stem cell transplantation for specific cancer types to improve outcomes.
Summary of Other Choices:
A: Incorrect. It is not necessary to use autologous stem cell transplantation solely for high-dose therapy. The listed cancers are not the most common indications for this procedure.
C: Incorrect. The statement about the graft-versus-tumor effect is misleading, and the listed cancers are not the most common ones treated with autologous stem cell transplantation.
D: Incorrect. Autologous stem cell transplantation is not used to shorten treatment or avoid extensive chemotherapy. The listed cancers