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

It requires factor IX replacement

Answer

It is an X-linked recessive disorder

Answer

It always presents with hemarthrosis

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

15 minutes

Answer

30 minutes

Answer

12 hours

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

Hyperhemolytic crisis

Answer

Aplastic crisis

Answer

Acute chest syndrome

Question 4

Desmopressin (DDAVP) is used in mild hemophilia A because it:

Accepted Answer

Stimulates the release of endogenous factor VIII and vWF

Answer

Provides exogenous factor VIII directly

Answer

Reverses the effects of anticoagulants

Answer

Increases platelet production

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

Engraftment failure

Answer

Sinusoidal obstruction syndrome

Answer

Cytomegalovirus infection

Question 6

Which laboratory finding would the nurse expect in iron-deficiency anemia?

Accepted Answer

Low MCV, low serum ferritin, elevated TIBC

Answer

Elevated MCV and elevated ferritin

Answer

Normal MCV and low reticulocyte count

Answer

Elevated direct bilirubin and spherocytes