CPHON Pediatric Blood Disorders and Hematology 1 — Questions and Answers
Question 1: A child with sickle cell disease presents with severe bone pain, fever, and chest infiltrates. The nurse recognizes this as:
- Aplastic crisis
- Acute chest syndrome (Correct answer)
- Splenic sequestration
- Vaso-occlusive crisis
Correct answer: Acute chest syndrome
Acute chest syndrome in sickle cell disease is defined by a new pulmonary infiltrate with fever, chest pain, and respiratory symptoms.
Question 2: Which blood product is used FIRST-LINE for a child with immune thrombocytopenic purpura (ITP) who has intracranial bleeding?
- Packed red blood cells
- Platelet transfusion (Correct answer)
- Fresh frozen plasma
- Cryoprecipitate
Correct answer: Platelet transfusion
Platelet transfusion is given urgently in ITP with life-threatening bleeding such as intracranial hemorrhage to raise the platelet count rapidly.
Question 3: A child with hemophilia A needs factor replacement. Which blood product is most appropriate?
- Fresh frozen plasma
- Factor VIII concentrate (Correct answer)
- Cryoprecipitate alone
- Vitamin K injection
Correct answer: Factor VIII concentrate
Factor VIII concentrate directly replaces the deficient clotting factor in hemophilia A and is more effective and precise than FFP.
Question 4: A 6-year-old with beta-thalassemia major requires chronic transfusion therapy. Which complication must the nurse monitor for over time?
- Hypocalcemia
- Iron overload (hemosiderosis) (Correct answer)
- Vitamin B12 deficiency
- Hypokalemia
Correct answer: Iron overload (hemosiderosis)
Repeated transfusions in thalassemia major lead to iron overload, damaging the heart, liver, and endocrine organs.
Question 5: Which finding is characteristic of disseminated intravascular coagulation (DIC) in a pediatric oncology patient?
- Elevated platelet count and normal PT
- Prolonged PT/PTT, low fibrinogen, and thrombocytopenia (Correct answer)
- Isolated neutropenia
- Elevated hemoglobin and hematocrit
Correct answer: Prolonged PT/PTT, low fibrinogen, and thrombocytopenia
DIC is characterized by simultaneous clotting and bleeding, resulting in consumption of clotting factors and platelets, causing prolonged PT/PTT and low fibrinogen.
Question 6: A child with aplastic anemia has an ANC of 150 cells/mm³. The nurse should place the child in:
- Contact isolation
- Droplet precautions
- Protective (reverse) isolation (Correct answer)
- Standard precautions only
Correct answer: Protective (reverse) isolation
An ANC below 500 cells/mm³ indicates severe neutropenia; protective isolation minimizes exposure to pathogens in this immunocompromised child.
CPHON Pediatric Blood Disorders and Hematology 1
A child with sickle cell disease presents with severe bone pain, fever, and chest infiltrates.
The nurse recognizes this as: