CPHON Pediatric Blood Disorders and Hematology 2

Question 1

Which statement about von Willebrand disease (vWD) Type 1 is correct?

Accepted Answer

It is characterized by partial quantitative deficiency of vWF

Answer

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:

Accepted Answer

2–4 hours

Answer

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:

Accepted Answer

Splenic sequestration crisis

Answer

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:

Accepted Answer

Stimulates the release of endogenous factor VIII and vWF

Answer

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:

Accepted Answer

Graft-versus-host disease (GVHD)

Answer

Classic acute GVHD presents with the triad of skin rash, diarrhea, and liver dysfunction (elevated bilirubin) after allogeneic transplant.

CPHON Exam Practice Test

CPHON Exam Practice Test

CPHON Pediatric Blood Disorders and Hematology 2