Nokea
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: The correct answer is B because autologous hematopoietic stem cell transplantation should be used when it can provide a survival benefit over chemotherapy. This approach is particularly beneficial for certain cancers such as neuroblastoma and relapsed lymphoma.
- Choice A is incorrect because the indication for autologous transplantation is not solely based on the need for high-dose therapy, but rather on providing a meaningful survival benefit over chemotherapy. The listed cancers are not the most common ones treated with this approach.
- Choice C is incorrect because the graft-versus-tumor effect is actually desired in some cases of autologous transplantation, and the listed cancers are not the most common ones treated with this approach.
- Choice D is incorrect because the purpose of autologous transplantation is not to avoid extensive chemotherapy but to provide a survival benefit. The listed cancers are not the most common ones treated with this approach.
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Hepato-splenomegaly with lymphadenopathy occur in all except:
- A. ALL
- B. lymphoma
- C. CML
- D. dissiminated TB
Correct Answer: C
Rationale: Step-by-step rationale:
1. Chronic Myeloid Leukemia (CML) primarily involves the bone marrow and blood, not the liver, spleen, or lymph nodes.
2. Hepato-splenomegaly with lymphadenopathy are commonly seen in Acute Lymphoblastic Leukemia (ALL) and lymphoma.
3. Disseminated TB can cause hepato-splenomegaly but typically does not present with lymphadenopathy.
Therefore, C (CML) is the correct answer as it does not typically present with the described findings.
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. This finding is concerning because supraclavicular nodes are not normally palpable. Enlarged supraclavicular nodes can indicate metastatic cancer. B is incorrect because mobile and nontender axillary nodes are usually benign. C is incorrect as the inability to palpate superficial nodes may be normal. D is incorrect as firm inguinal nodes could indicate a localized infection or inflammation.
Graft versus host disease is a complication of transfusion in patients of bone marrow transplantation. This can be best avoided by
- A. Transfusion of packed cells
- B. Irradiating the blood components before transfusion
- C. Transfusing fresh whole blood
- D. Replacement by crystalloids
Correct Answer: B
Rationale: The correct answer is B: Irradiating the blood components before transfusion. Graft versus host disease occurs when donor T-cells attack the recipient's tissues in bone marrow transplant patients. Irradiating blood components helps to deactivate T-cells in the donor blood, preventing them from attacking the recipient's tissues. Transfusion of packed cells (A) or fresh whole blood (C) does not address the issue of T-cell activation. Replacement by crystalloids (D) is not a solution for preventing graft versus host disease.
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
A 13-year-old boy presents to the emergency department with complaints of headache and visual changes. History reveals progressive dyspnea on exertion, generalized fatigue, and increased bruising. His labs are significant for a WBC of 350,000/mcL, of which 80% are reported to be blasts and appear to be myeloblasts without the presence of Auer rods. His hemoglobin is 7.2 g/dL, and his platelets are 18,000/mcL. A CT scan of the head shows a small intracerebral hemorrhage. His coags are normal. Which of the following is the most appropriate therapy?
- A. Start induction chemotherapy.
- B. Perform emergent leukapheresis followed the next day by induction chemotherapy.
- C. Perform emergent leukapheresis plus hydroxyurea.
- D. Provide emergent cranial radiation plus hydroxyurea followed the next day by induction chemotherapy.
Correct Answer: C
Rationale: The correct answer is C: Perform emergent leukapheresis plus hydroxyurea. In this scenario, the patient presents with symptoms suggestive of acute myeloid leukemia with hyperleukocytosis and intracerebral hemorrhage. The goal of emergent therapy is to rapidly reduce the high blast count to prevent further complications such as leukostasis and hemorrhage. Leukapheresis can provide immediate reduction in the blast count, while hydroxyurea can further decrease the WBC count. Starting induction chemotherapy immediately can lead to tumor lysis syndrome due to rapid cell destruction. Emergent cranial radiation is not the primary intervention for hyperleukocytosis. Performing leukapheresis alone without adjunctive therapy like hydroxyurea may not adequately control the blast count. Thus, the most appropriate initial therapy is emergent leukapheresis plus hydroxyurea to stabilize the patient before initiating induction chemotherapy.