Nokea
Incorrect about pernicious an:
- A. hyperchlorhydria
- B. premature graying of hair
- C. anti intrinsic factor antibody in 60% of pts.
- D. gastric polyp may occur
Correct Answer: A
Rationale: The correct answer is A, hyperchlorhydria. Pernicious anemia is characterized by vitamin B12 deficiency due to lack of intrinsic factor. Hyperchlorhydria refers to high stomach acid production, not a symptom of pernicious anemia. Premature graying of hair and gastric polyp are potential features of pernicious anemia. Anti intrinsic factor antibody presence in 60% of patients is a characteristic finding in pernicious anemia.
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An 8-year-old girl presents with National Cancer Institute (NCI) Standard Risk acute pre-B-cell acute lymphoblastic leukemia. Her family history is significant for her mother having been diagnosed with breast cancer at age 34 years and a maternal uncle who developed osteosarcoma as a teenager. What cytogenetic abnormality is most likely to be detected in this patient?
- A. t(1;19)
- B. CRLF2 rearrangement with a JAK2 mutation
- C. KMT24 rearrangement
- D. Hypodiploidy with a modal chromosome number of 34
Correct Answer: D
Rationale: The correct answer is D: Hypodiploidy with a modal chromosome number of 34. In pediatric pre-B-cell acute lymphoblastic leukemia, hypodiploidy (fewer than 44 chromosomes) is associated with poor prognosis. A modal chromosome number of 34 is specifically linked to high-risk disease.
A: t(1;19) is characteristic of pre-B-cell acute lymphoblastic leukemia but is associated with intermediate prognosis, not standard risk.
B: CRLF2 rearrangement with a JAK2 mutation is seen in a subset of high-risk acute lymphoblastic leukemia cases, not standard risk.
C: KMT24 rearrangement is not a known cytogenetic abnormality in acute lymphoblastic leukemia.
The nurse is concerned that a patient is demonstrating signs of red blood cell production. What laboratory value did the nurse most likely use to make this decision?
- A. Iron
- B. Bilirubin
- C. Thrombin
- D. Intrinsic factor
Correct Answer: B
Rationale: The correct answer is B: Bilirubin. Bilirubin is a product of red blood cell breakdown and its levels can indicate the rate of red blood cell production. Elevated bilirubin can suggest increased RBC destruction or hemolysis. Iron (A) is related to anemia but not a direct indicator of RBC production. Thrombin (C) is a clotting factor, not related to RBC production. Intrinsic factor (D) is involved in vitamin B12 absorption, not directly linked to RBC production.
An 18-year old male patient presents with bruising, fatigue, and diffuse extremity pain. He is noted to be tachypneic and hypoxic and has a diffuse interstitial infiltrate on chest x-ray. CBC reveals a WBC count of 285,000/mm3 (85% myeloblasts, with monocytic morphology), hemoglobin of 7.9 g/dL, and platelet count of 36,000/mm3. What is the most likely cause of the infiltrate and respiratory symptoms and the most appropriate initial treatment?
- A. Hyperleukocytosis; initiation of induction chemotherapy
- B. Hyperleukocytosis; leukapheresis or manual exchange transfusion and initiation of induction chemotherapy
- C. COVID-19 infection; convalescent plasma and prednisone
- D. Pneumococcal pneumonia; vancomycin
Correct Answer: B
Rationale: The correct answer is B. The patient's presentation with tachypnea, hypoxia, and diffuse interstitial infiltrate on chest x-ray suggests leukostasis syndrome due to hyperleukocytosis. The extremely high WBC count of 285,000/mm3 with myeloblasts indicates acute myeloid leukemia. Leukapheresis or manual exchange transfusion is needed to rapidly reduce the number of leukemic blasts in circulation to prevent complications like tissue hypoxia. Initiation of induction chemotherapy is essential for long-term management of AML.
Choice A is incorrect because induction chemotherapy alone may not rapidly reduce the WBC count in cases of leukostasis. Choice C is incorrect as the patient's clinical scenario is not consistent with COVID-19 infection, and convalescent plasma is not indicated for leukostasis. Choice D is incorrect as the patient's symptoms are not typical for pneumococcal pneumonia, and vancomycin is not the initial
You have been asked to see a 15-year-old girl who is being referred for evaluation of an ovarian mass. Her history is also significant for secondary amenorrhea, and physical examination shows signs of virilization. As you review her family history, what syndrome will you consider?
- A. Li-Fraumeni syndrome
- B. DICER-1 syndrome
- C. Turner syndrome
- D. Beckwith-Wiedemann syndrome
Correct Answer: B
Rationale: The correct answer is B: DICER-1 syndrome. This syndrome is associated with ovarian tumors, secondary amenorrhea, and signs of virilization due to androgen-secreting tumors. Li-Fraumeni syndrome (A) is characterized by multiple primary cancers but not specifically ovarian masses. Turner syndrome (C) presents with ovarian dysgenesis and primary amenorrhea. Beckwith-Wiedemann syndrome (D) is associated with overgrowth and abdominal wall defects, not ovarian masses. In this case, the presence of ovarian mass, secondary amenorrhea, and virilization point towards DICER-1 syndrome.
An 18-year old male patient with acute lymphoblastic leukemia recently started maintenance therapy and is complaining of increased hip pain. The pain increases during weight-bearing activity; however, it occasionally hurts at night as well. His CBCd is normal. Which of the following risk factors is most commonly associated with this process?
- A. Younger age at diagnosis
- B. Non-White race
- C. Low body-mass index
- D. Dexamethasone exposure
Correct Answer: D
Rationale: The correct answer is D: Dexamethasone exposure. Dexamethasone is a corticosteroid commonly used in the treatment of acute lymphoblastic leukemia. It can cause avascular necrosis of the hip due to its effect on bone metabolism, leading to increased hip pain during weight-bearing activities. This is a known side effect seen more commonly in patients exposed to dexamethasone.
Incorrect Choices:
A: Younger age at diagnosis - Age is not a known risk factor for avascular necrosis in this context.
B: Non-White race - Race is not a known risk factor for avascular necrosis in this context.
C: Low body-mass index - Body mass index is not a known risk factor for avascular necrosis in this context.