Nokea
Koilonychias is a specific feature of
- A. Hemolytic anemia
- B. Sickle cell disease
- C. Thalassemia major
- D. Iron deficiency anemia
Correct Answer: D
Rationale: Step-by-step rationale for why the correct answer is D (Iron deficiency anemia):
1. Koilonychias is characterized by spoon-shaped nails, a classic sign of iron deficiency anemia due to changes in nail structure.
2. Iron deficiency leads to decreased production of keratin, causing nail deformities like koilonychias.
3. Hemolytic anemia (choice A) and Sickle cell disease (choice B) are not typically associated with koilonychias.
4. Thalassemia major (choice C) can cause nail changes, but koilonychias is more commonly seen in iron deficiency anemia.
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A 12-year-old patient has been referred to you following complete resection with clean margins of a high-grade malignant peripheral nerve sheath tumor of the shoulder region. The tumor measured approximately 4 cm in greatest dimension. A CT scan of the chest and a bone scan were within normal limits. The patient does not have evidence of neurofibromatosis type 1 (NF1). Which of the following treatment approaches would you recommend?
- A. Chemotherapy with doxorubicin and ifosfamide
- B. Radiotherapy
- C. Chemotherapy with doxorubicin and ifosfamide plus radiotherapy
- D. Observation
Correct Answer: D
Rationale: The correct answer is D: Observation. In this case, the patient has undergone complete resection of the tumor with clean margins and there is no evidence of metastasis or neurofibromatosis type 1. Given these factors, observation is the most appropriate approach as there is no indication for adjuvant chemotherapy or radiotherapy. Chemotherapy with doxorubicin and ifosfamide (Choice A) is typically reserved for cases with high-risk features such as incomplete resection or metastasis. Radiotherapy (Choice B) may be considered in certain cases, but in this scenario where surgery was curative and there is no evidence of spread, it is not necessary. Chemotherapy plus radiotherapy (Choice C) may be overly aggressive and carry unnecessary risks for a patient who has already had successful surgery. Thus, observation is the most appropriate approach in this situation.
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
Which statement is correct regarding lymphocyte counts in infants versus adults?
- A. NK-cell numbers are lowest at birth and increase with age.
- B. B-cell numbers are highest at birth and decline with age.
- C. T-cell numbers in infants are higher than in adults.
- D. Infants have low lymphocyte counts that increase with age.
Correct Answer: C
Rationale: The correct answer is C because T-cell numbers in infants are indeed higher than in adults. This is due to the active development of the immune system in infants, leading to a higher proportion of T-cells.
A is incorrect because NK-cell numbers in infants are actually higher at birth and decrease with age.
B is incorrect because B-cell numbers are lower at birth and increase with age.
D is incorrect because infants do not have low lymphocyte counts; their immune system is actively developing, leading to higher lymphocyte counts compared to adults.
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
The mean cell volume of the patient is 90 fl and the reticulocyte production index is low. Which of the following is the most possible cause of anemia in this patient?
- A. Thalassemia
- B. Vitamin B12 deficiency
- C. Aplastic anemia
- D. Sideroblastic anemia
Correct Answer: C
Rationale: The correct answer is C: Aplastic anemia. Aplastic anemia is characterized by a low reticulocyte production index due to bone marrow failure, leading to decreased red blood cell production and subsequently causing anemia. Thalassemia (choice A) is a genetic disorder resulting in abnormal hemoglobin production, not bone marrow failure. Vitamin B12 deficiency (choice B) can lead to macrocytic anemia, not a low reticulocyte production index. Sideroblastic anemia (choice D) is characterized by abnormal iron metabolism in the bone marrow, not bone marrow failure.