CPEN Hematology and Oncology Emergencies — Questions and Answers
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?
- Community-acquired pneumonia
- Acute chest syndrome (Correct answer)
- Pulmonary embolism
- Spontaneous pneumothorax
Correct answer: Acute chest syndrome
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?
- Schedule urgent outpatient follow-up with oncology within 24 hours
- Administer broad-spectrum IV antibiotics within 60 minutes of triage (Correct answer)
- Draw blood cultures and wait for results before initiating antibiotics
- Administer oral antibiotics and discharge with strict return precautions
Correct answer: Administer broad-spectrum IV antibiotics within 60 minutes of triage
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?
- Immobilize the joint, apply ice, and observe for 24 hours
- Administer factor VIII concentrate immediately (Correct answer)
- Give ibuprofen for pain control and arrange outpatient follow-up
- Perform joint aspiration to relieve pressure
Correct answer: Administer factor VIII concentrate immediately
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?
- Hypertensive encephalopathy from corticosteroid use
- Leukostasis syndrome (Correct answer)
- Tumor lysis syndrome
- Cerebral venous sinus thrombosis
Correct answer: Leukostasis syndrome
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?
- Adrenal crisis
- Tumor lysis syndrome (Correct answer)
- Septic shock
- Rhabdomyolysis
Correct answer: Tumor lysis syndrome
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.
Question 6: A 6-year-old presents with pallor, fatigue, and petechiae. CBC shows hemoglobin 5.8 g/dL, platelets 14,000/μL, and WBC 48,000/μL. Which diagnosis is most consistent with this presentation?
- Severe iron deficiency anemia
- Immune thrombocytopenic purpura (ITP)
- Acute leukemia (Correct answer)
- Aplastic anemia
Correct answer: Acute leukemia
The combination of anemia, thrombocytopenia, markedly elevated WBC, and constitutional symptoms in a child strongly suggests acute leukemia. In ITP, only platelets are depressed. In iron deficiency anemia, only hemoglobin is low. Aplastic anemia causes pancytopenia but the WBC is typically low, not elevated. Bone marrow biopsy confirms leukemia.
CPEN Hematology and Oncology Emergencies
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?