Nokea
A 5-year-old boy is evaluated for apparent ongoing hemolysis. His hemoglobin is 9.5 g/dL, with 8% reticulocytes and MCV 87 fL. Platelets and leukocytes are normal. His direct antiglobulin test (DAT) is negative. No cold agglutinin is detectable. His family history is negative for blood disorders. Peripheral smear reveals basophilic stippling in 10% of the red blood cells. Given these findings, which of the following blood disorders is most likely?
- A. Rh-null disease
- B. Hereditary pyropoikilocytosis
- C. Glucose phosphate isomerase deficiency
- D. Unstable hemoglobin
Correct Answer: E
Rationale: I'm sorry, but it seems like there might be a mistake in the question as there is no option "E" provided. Could you please provide the correct answer choice so that I can give you a step-by-step rationale for why that answer is correct?
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Henoch Schonlein purpura is not associated with:
- A. thrombocytopenia
- B. palpable purpura
- C. intussusception
- D. acute diffuse glomerulonephritis
Correct Answer: A
Rationale: Henoch Schonlein purpura is characterized by palpable purpura, abdominal pain, arthritis, and acute diffuse glomerulonephritis. Thrombocytopenia is not typically associated with Henoch Schonlein purpura, making choice A correct. Thrombocytopenia is more commonly seen in conditions like immune thrombocytopenic purpura. Choices B, C, and D are incorrect as palpable purpura, intussusception, and acute diffuse glomerulonephritis are commonly associated with Henoch Schonlein purpura.
A 16-year-old female presents to the emergency room with a new complaint of chest pain. When performing a review of systems and physical examination, which of the following would substantially decrease your suspicion for a diagnosis of pulmonary embolism?
- A. Cough
- B. Fever
- C. Rib tenderness
- D. Shortness of breath
Correct Answer: C
Rationale: The correct answer is C: Rib tenderness. Rib tenderness is a symptom more commonly associated with musculoskeletal issues rather than pulmonary embolism. Pulmonary embolism typically presents with symptoms such as chest pain, shortness of breath, and sometimes cough. Fever is not a typical symptom of pulmonary embolism. Shortness of breath is a common symptom but not specific enough to substantially decrease suspicion for pulmonary embolism.
A nurse is administering epoetin intravenously to a client who has renal failure. Which of the following actions should the nurse take?
- A. Shake the vial before using.
- B. Administer via IV bolus over 1 to 3 min.
- C. Dilute the drug first with D5W.
- D. Save the used vial for the next dose.
Correct Answer: B
Rationale: The correct answer is B: Administer via IV bolus over 1 to 3 min. This route and method of administration is appropriate for epoetin to ensure proper absorption and effect. Shaking the vial (A) can denature the drug. Diluting with D5W (C) is not recommended as it may alter the drug's effectiveness. Saving the used vial for the next dose (D) is unsafe and violates infection control practices.
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.
You are consulting on a 10-year-old male with severe persistent neutropenia, a history of recurrent infections, and warts. The rest of the peripheral blood count is normal. His mother also has neutropenia. Bone marrow examination shows a hypercellular marrow and retained myeloid cells with vacuolated cytoplasm. There are no abnormalities in the red cells or platelet precursors. Cytogenetics are 46XY. You start granulocyte colony stimulating factor therapy and the neutrophil count increases. A mutation in which of the following genes is most likely to have caused this familial inherited bone marrow failure syndrome?
- A. CXCR4
- B. ELANE
- C. GATA 2
- D. Mitochondrial DNA
Correct Answer: A
Rationale: The correct answer is A: CXCR4. In this case, the patient presents with severe neutropenia, recurrent infections, and warts, suggestive of WHIM syndrome, where CXCR4 mutations are often involved. CXCR4 plays a crucial role in immune cell trafficking and retention in the bone marrow. The hypercellular marrow and vacuolated myeloid cells are consistent with WHIM syndrome. The absence of abnormalities in red cells or platelet precursors rules out other syndromes. Mutations in ELANE are commonly associated with congenital neutropenia, not familial inherited bone marrow failure syndromes. GATA2 mutations are linked to familial myelodysplastic syndromes, not typically presenting with neutropenia and warts. Mitochondrial DNA mutations are more related to mitochondrial disorders, which usually manifest with multi-system involvement, not specific to bone marrow failure syndromes.