CPEN Hematology and Oncology Emergencies

Question 1

A 7-year-old with sickle cell disease presents with fever, tachypnea, severe chest pain, and a new pulmonary infiltrate on chest X-ray. What is the most likely diagnosis?

Accepted Answer

Acute chest syndrome

Answer

Acute chest syndrome (ACS) is a life-threatening complication of sickle cell disease defined by a new pulmonary infiltrate plus at least one of: chest pain, fever, or respiratory symptoms. It results from vaso-occlusion and fat embolism in the pulmonary vasculature. Treatment includes supplemental oxygen, IV fluids, analgesia, antibiotics, and often exchange transfusion.

Question 2

A 4-year-old undergoing chemotherapy presents with a temperature of 38.6°C and an absolute neutrophil count (ANC) of 280 cells/μL. What is the priority nursing action?

Accepted Answer

Administer broad-spectrum IV antibiotics within 60 minutes of triage

Answer

Febrile neutropenia (fever + ANC <500 cells/μL) in a pediatric oncology patient is a medical emergency with significant mortality. Broad-spectrum IV antibiotics (e.g., cefepime or piperacillin-tazobactam) must be administered within 60 minutes of presentation. Blood cultures are drawn first but must not delay antibiotic initiation.

Question 3

A 2-year-old with hemophilia A presents after a minor fall with a painful, swollen knee hemarthrosis. What is the first-line treatment?

Accepted Answer

Administer factor VIII concentrate immediately

Answer

Hemophilia A is a factor VIII deficiency. Acute hemarthrosis requires immediate factor VIII replacement to arrest bleeding. NSAIDs like ibuprofen are contraindicated in hemophilia because they inhibit platelet aggregation and worsen bleeding. Joint aspiration without prior factor replacement is also contraindicated due to the risk of uncontrolled hemorrhage.

Question 4

A 5-year-old with leukemia presents with confusion, severe headache, and a blood pressure of 182/112 mmHg. The WBC is 380,000/μL. Which complication is most likely responsible?

Accepted Answer

Leukostasis syndrome

Answer

Leukostasis occurs when an extremely elevated WBC (typically >100,000/μL) causes hyperviscosity and vascular sludging in the microcirculation. In the CNS, this produces headache, confusion, and focal deficits. In the lungs, it causes respiratory failure. This is a hematologic emergency requiring emergent cytoreduction with leukapheresis or hydroxyurea.

Question 5

A child with ALL (acute lymphoblastic leukemia) presents after intensified chemotherapy with weakness, peaked T waves on ECG, and oliguria. Labs show: K+ 6.9 mEq/L, phosphate 8.4 mg/dL, calcium 6.0 mg/dL, uric acid 13 mg/dL. Which syndrome does this represent?

Accepted Answer

Tumor lysis syndrome

Answer

Tumor lysis syndrome (TLS) results from rapid destruction of malignant cells releasing intracellular contents into the bloodstream. The classic electrolyte pattern is hyperkalemia, hyperphosphatemia, hypocalcemia, and hyperuricemia. This can cause fatal arrhythmias and acute renal failure. Treatment includes aggressive hydration, allopurinol or rasburicase, and electrolyte correction.

CPEN - Certified Pediatric Emergency Nurse Practice Test

CPEN - Certified Pediatric Emergency Nurse Practice Test

CPEN Hematology and Oncology Emergencies