A 19-year old male patient with a history of acute lymphoblastic leukemia, currently 13 years from completion of therapy, presents for a fertility consultation. He is interested in his risk for infertility. Which of the following statements is true?
- A. A semen analysis at this point would provide accurate information about future fertility.
- B. Males can maintain gonadal function at higher cumulative alkylator dosages compared with females.
- C. He should have been offered sperm cryopreservation at diagnosis.
- D. His risk for testosterone deficiency is greater than his risk for infertility.
Correct Answer: A
Rationale: Step 1: Semen analysis is the gold standard for assessing male fertility.
Step 2: The patient's history of ALL and completion of therapy make semen analysis relevant.
Step 3: Long-term survivors of ALL are at risk for infertility due to treatment effects.
Step 4: Semen analysis can provide valuable information on sperm count, motility, and morphology.
Summary:
- Option A is correct as semen analysis is crucial for assessing male fertility.
- Option B is incorrect as alkylator dosages affect both males and females.
- Option C is incorrect as sperm cryopreservation should ideally be offered at diagnosis.
- Option D is incorrect as infertility risk may be higher than testosterone deficiency in this case.
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The patient is a 2-month-old boy who presented with a skin abscess and is febrile. On exam, he is noted to have silvery hair and hypopigmented skin. A CBC shows a leukocyte count of 3.4 K/mcL with 10% neutrophils. What does the abnormality on the peripheral smear suggest?
- A. Abnormal lysosomal biogenesis
- B. Abnormal ribosome function
- C. Abnormal phagocytosis of opsonized particles
- D. Abnormal mitochondrial activity
Correct Answer: A
Rationale: The correct answer is A: Abnormal lysosomal biogenesis. The patient's presentation of silvery hair and hypopigmented skin suggests a lysosomal storage disorder like Chediak-Higashi syndrome. The peripheral smear showing 10% neutrophils with a low leukocyte count indicates impaired neutrophil function due to abnormal lysosomal biogenesis. This affects phagocytosis and intracellular killing of pathogens, leading to recurrent infections and abscess formation.
Incorrect choices:
B: Abnormal ribosome function - not related to the patient's presentation.
C: Abnormal phagocytosis of opsonized particles - the primary issue is with lysosomal biogenesis, not phagocytosis.
D: Abnormal mitochondrial activity - does not explain the silvery hair and hypopigmented skin seen in Chediak-Higashi syndrome.
Which of the following is wrong concerning Philadelphia chromosome:
- A. shortening of long arm of chromosome 22
- B. Philadelphia:-ve cases have bad prognosis
- C. diagnostic of CML
- D. found in lymphocytes
Correct Answer: D
Rationale: The correct answer is D, as the Philadelphia chromosome is typically found in myeloid cells, not lymphocytes. This is because the Philadelphia chromosome results from a translocation between chromosomes 9 and 22, leading to the formation of the BCR-ABL fusion gene, which is associated with chronic myeloid leukemia (CML). Choice A is correct as the Philadelphia chromosome results from the shortening of the long arm of chromosome 22. Choice B is incorrect as Philadelphia positivity in CML patients is associated with a worse prognosis. Choice C is correct as the presence of the Philadelphia chromosome is a diagnostic hallmark of CML.
The pathophysiology of venous thrombosis is often explained by Virchow's triad, which includes hypercoagulability, endothelial injury, and venous stasis. Based on Virchow's triad and your knowledge of risk factors for thrombosis, which of the following pediatric patients has the greatest risk of hospital-acquired venous thromboembolism?
- A. 3-day-old full-term infant admitted to hospital pediatrics for hyperbilirubinemia
- B. 6-month-old male admitted to the infectious disease unit for respiratory syncytial virus
- C. Ex-28 week premature infant, requiring NICU-level care for necrotizing enterocolitis
- D. 7-year-old male with acute lymphoblastic leukemia receiving maintenance chemotherapy admitted to hematology/oncology unit for fever and neutropenia
Correct Answer: C
Rationale: The correct answer is C because the ex-28 week premature infant requiring NICU-level care for necrotizing enterocolitis has the greatest risk of hospital-acquired venous thromboembolism. Premature infants have inherent hypercoagulability due to immature coagulation factors and increased risk of endothelial injury from central lines or catheters. Necrotizing enterocolitis further increases the risk of venous stasis due to decreased gut perfusion.
Choice A: The 3-day-old full-term infant admitted for hyperbilirubinemia is less likely to have significant risk factors for thrombosis compared to a premature infant with necrotizing enterocolitis.
Choice B: The 6-month-old male admitted for respiratory syncytial virus is less likely to have prolonged immobilization or other significant risk factors compared to a premature infant in the NICU.
Choice D: The 7-year-old male with acute lymphoblastic leukemia has a higher risk of thromb
A 17-year-old female presents with cervical adenopathy and a history of daily fevers and drenching night sweats. A biopsy is performed and reveals classic Hodgkin lymphoma. Which of the following is least appropriate as part of the staging workup?
- A. Chest x-ray
- B. CT scan of chest, abdomen, and pelvis
- C. Functional imaging (PET scan)
- D. Lumbar puncture and cerebrospinal fluid (CSF) analysis
Correct Answer: D
Rationale: The correct answer is D: Lumbar puncture and cerebrospinal fluid (CSF) analysis. In Hodgkin lymphoma staging, CNS involvement is rare at initial presentation. Therefore, routine CSF analysis is not recommended unless there are specific neurological symptoms suggestive of CNS disease. A lumbar puncture carries risks of complications and should not be performed routinely. Choices A, B, and C are appropriate for staging in Hodgkin lymphoma to evaluate for distant metastases and characterize disease extent.
In a study to investigate the rates of central line–acquired bacterial infections, it is discovered that patient length of stay (LOS) is not normally distributed but is highly right-skewed. What is the correct relationship between the mean, median, and mode of LOS?
- A. The mean is less than the median but greater than the mode.
- B. The mean is equal to the median and the mode.
- C. The mean is greater than the median and mode.
- D. The mean and median will both be less than the mode.
Correct Answer: C
Rationale: The correct answer is C: The mean is greater than the median and mode. In a highly right-skewed distribution, the mean is pulled towards the higher end by the extreme values, making it greater than the median, which is the middle value. The mode is the most frequent value, which is typically lower than the mean in a right-skewed distribution. In summary, the mean is influenced by extreme values, causing it to be greater than both the median and the mode in a highly right-skewed distribution.