CPHON Pediatric Blood Disorders and Hematology 2 — Questions and Answers
Question 1: Which statement about von Willebrand disease (vWD) Type 1 is correct?
- It requires factor IX replacement
- It is characterized by partial quantitative deficiency of vWF (Correct answer)
- It is an X-linked recessive disorder
- It always presents with hemarthrosis
Correct answer: It is characterized by partial quantitative deficiency of vWF
Type 1 vWD is the most common form and involves a partial reduction in the quantity of von Willebrand factor, inherited in an autosomal dominant pattern.
Question 2: A child with a hemoglobin of 6.5 g/dL is scheduled for a packed red blood cell transfusion. The nurse should transfuse over:
- 15 minutes
- 30 minutes
- 2–4 hours (Correct answer)
- 12 hours
Correct answer: 2–4 hours
Packed red blood cells should be transfused over 2–4 hours to avoid fluid overload and allow physiologic adaptation, especially in children.
Question 3: The nurse notes a child with sickle cell disease has a spleen that is markedly enlarged and the hemoglobin dropped from 9 to 5 g/dL acutely. This presentation suggests:
- Hyperhemolytic crisis
- Splenic sequestration crisis (Correct answer)
- Aplastic crisis
- Acute chest syndrome
Correct answer: Splenic sequestration crisis
Splenic sequestration crisis involves rapid pooling of blood in the spleen causing acute anemia, splenomegaly, and circulatory compromise.
Question 4: Desmopressin (DDAVP) is used in mild hemophilia A because it:
- Provides exogenous factor VIII directly
- Stimulates the release of endogenous factor VIII and vWF (Correct answer)
- Reverses the effects of anticoagulants
- Increases platelet production
Correct answer: Stimulates the release of endogenous factor VIII and vWF
DDAVP stimulates endothelial cells to release stored factor VIII and vWF, temporarily raising their plasma levels in mild cases.
Question 5: A child undergoing allogeneic stem cell transplant develops a new rash, diarrhea, and elevated bilirubin on day +30. The nurse suspects:
- Engraftment failure
- Graft-versus-host disease (GVHD) (Correct answer)
- Sinusoidal obstruction syndrome
- Cytomegalovirus infection
Correct answer: Graft-versus-host disease (GVHD)
Classic acute GVHD presents with the triad of skin rash, diarrhea, and liver dysfunction (elevated bilirubin) after allogeneic transplant.
Question 6: Which laboratory finding would the nurse expect in iron-deficiency anemia?
- Elevated MCV and elevated ferritin
- Low MCV, low serum ferritin, elevated TIBC (Correct answer)
- Normal MCV and low reticulocyte count
- Elevated direct bilirubin and spherocytes
Correct answer: Low MCV, low serum ferritin, elevated TIBC
Iron-deficiency anemia causes microcytic (low MCV) anemia, depleted iron stores (low ferritin), and increased iron-binding capacity (elevated TIBC).
CPHON Pediatric Blood Disorders and Hematology 2
Which statement about von Willebrand disease (vWD) Type 1 is correct?