Pediatric Blood Disorders and Hematology Flashcards
6 cards from real CPHON practice questions. Tap to flip, then mark Knew It or Still Learning — missed cards come back until you master them.
Read the first 6 Pediatric Blood Disorders and Hematology flashcards as text
Which statement about von Willebrand disease (vWD) Type 1 is 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.
A child with a hemoglobin of 6.5 g/dL is scheduled for a packed red blood cell transfusion. The nurse should transfuse over:
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.
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:
Answer: Splenic sequestration crisis
Splenic sequestration crisis involves rapid pooling of blood in the spleen causing acute anemia, splenomegaly, and circulatory compromise.
Desmopressin (DDAVP) is used in mild hemophilia A because it:
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.
A child undergoing allogeneic stem cell transplant develops a new rash, diarrhea, and elevated bilirubin on day +30. The nurse suspects:
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.
Which laboratory finding would the nurse expect in iron-deficiency anemia?
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).