Hemolytic Anemia: Model Clinical Case and Viva Q&A

Clinical Case of Hemolytic Anemia in Children

A 4-year-old boy is brought to the pediatric emergency department by his parents with complaints of progressive pallor, fatigue, and yellowing of the eyes for the past week. The parents also noticed that his urine has become darker over the last few days.

History:

• No significant past medical history
• No recent infections or medication use
• No family history of anemia or jaundice

Physical Examination:

• Pale conjunctiva and mucous membranes
• Icteric sclera
• Mild hepatosplenomegaly
• Tachycardia (heart rate 120 bpm)
• No lymphadenopathy or rash

Laboratory Results:

• Hemoglobin: 7.2 g/dL (normal range: 11.5-13.5 g/dL)
• Reticulocyte count: 8% (normal range: 0.5-2.5%)
• Mean Corpuscular Volume (MCV): 85 fL (normal range: 75-87 fL)
• Total bilirubin: 3.8 mg/dL (normal range: 0.3-1.2 mg/dL)
• Direct bilirubin: 0.5 mg/dL (normal range: 0-0.3 mg/dL)
• Lactate dehydrogenase (LDH): 580 U/L (normal range: 110-295 U/L)
• Haptoglobin: < 10 mg/dL (normal range: 30-200 mg/dL)

Further Investigations:

• Peripheral blood smear: Spherocytes present
• Direct Coombs test: Negative
• Osmotic fragility test: Increased

Diagnosis:

Based on the clinical presentation and laboratory findings, the child is diagnosed with hereditary spherocytosis, a type of hemolytic anemia.

Management:

The child is admitted for further evaluation and management, including:

• Blood transfusion to correct severe anemia
• Folic acid supplementation
• Genetic counseling for the family
• Discussion of potential splenectomy in the future

Clinical Presentations of Hemolytic Anemia in Children

1. Acute Onset with Severe Symptoms:
   • Sudden pallor, fatigue, and shortness of breath
   • Jaundice and dark urine
   • Tachycardia and potential heart failure symptoms
   • Abdominal pain due to hepatosplenomegaly
2. Chronic, Mild Anemia with Intermittent Exacerbations:
   • Persistent mild pallor and fatigue
   • Intermittent jaundice
   • Growth retardation or delayed puberty
   • Recurrent gallstones
3. Neonatal Hemolytic Anemia:
   • Severe jaundice within 24-48 hours of birth
   • Hepatosplenomegaly
   • Risk of kernicterus if untreated
   • Hydrops fetalis in severe cases
4. Hemolytic Anemia Secondary to Infection:
   • Fever and other signs of infection
   • Acute onset of anemia symptoms
   • Possible hepatosplenomegaly
   • May be associated with specific infections (e.g., malaria, babesiosis)
5. Drug-Induced Hemolytic Anemia:
   • Onset of symptoms after starting new medication
   • Can be acute or gradual
   • May be accompanied by other drug reaction symptoms (e.g., rash)
6. Exercise-Induced Hemolytic Anemia:
   • Symptoms primarily after intense physical activity
   • Dark urine post-exercise
   • Fatigue and reduced exercise tolerance
7. Hemolytic Anemia with Thrombosis:
   • Signs of hemolysis along with thrombotic events
   • Can include stroke, pulmonary embolism, or deep vein thrombosis
   • Seen in conditions like paroxysmal nocturnal hemoglobinuria
8. Hemolytic Uremic Syndrome Presentation:
   • Triad of hemolytic anemia, thrombocytopenia, and acute kidney injury
   • Often preceded by bloody diarrhea
   • Pallor, petechiae, and oliguria/anuria

Knowledge Check: Question and Answers for Medical Students & Professionals

This interactive quiz component covers essential viva questions and answers. It includes 30 high-yield viva questions with detailed answers.

Question 1 of 30

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