Exam 4 Case Studies

1.1: A 12-year-old boy presents with fever, cough, chest pain, chronic leg ulcers, WBC 22.0, RBC 1.4, Hgb 4.0. What abnormal hematologic findings are present?

Severe normocytic anemia, leukocytosis, and sickle cell morphology (sickled cells + target cells).
Explanation:
• Hgb 4.0 = severe anemia
• Chronic leg ulcers + chest pain = classic for sickle cell
• WBC elevated due to underlying inflammation/infection
• Smear in this case shows sickled cells.

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1.2: A 12-year-old boy presents with fever, cough, chest pain, chronic leg ulcers, WBC 22.0, RBC 1.4, Hgb 4.0. What abnormal hematologic findings are present?

What is the most probable disorder?

Sickle Cell Disease (Hb SS).
Explanation: Symptoms + severe anemia + chronic ulcers + crisis → classic Hb SS picture.

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1.3: A 12-year-old boy presents with fever, cough, chest pain, chronic leg ulcers, WBC 22.0, RBC 1.4, Hgb 4.0. What abnormal hematologic findings are present?

What screening test should be recommended?

Hemoglobin solubility test (Sickle solubility test).
Explanation: Detects presence of Hb S polymerization in deoxygenated conditions.

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1.4: A 12-year-old boy presents with fever, cough, chest pain, chronic leg ulcers, WBC 22.0, RBC 1.4, Hgb 4.0. What abnormal hematologic findings are present?

If the solubility test is positive, what confirmatory test should follow?

Hemoglobin electrophoresis (alkaline + acid).
Explanation: Differentiates Hb S from other variants (D/G).

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1.5: A 12-year-old boy presents with fever, cough, chest pain, chronic leg ulcers, WBC 22.0, RBC 1.4, Hgb 4.0. What abnormal hematologic findings are present?

What result is expected on the confirmatory electrophoresis for SCD?

No Hb A, mostly Hb S, increased Hb F, increased A2.
Explanation: Hb SS has 0% Hb A, usually >80% S, with elevated F.

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1.6: A 12-year-old boy presents with fever, cough, chest pain, chronic leg ulcers, WBC 22.0, RBC 1.4, Hgb 4.0. What abnormal hematologic findings are present?

What is the cause of the elevated WBC count in this case?

Inflammation or infection associated with sickle crisis (acute chest syndrome).
Explanation: WBC commonly increases during vaso-occlusive episodes.

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2.1: A child’s alkaline electrophoresis shows two prominent bands: one in A position and one in S/D/G region. Solubility test is POSITIVE. What is the interpretation?

Sickle Cell Trait (Hb AS).
Explanation:
• Positive solubility = Hb S present
• Presence of BOTH A and S = trait
• Amount of A > S confirms AS.

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2.2: Acid electrophoresis confirms identity: S separates from D/G. What does this show?

The S band confirms Hb S (not D or G), consistent with Sickle Cell Trait.
Explanation: On acid gel:
• S moves uniquely
• D and G move differently, helping confirm S.

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2.3: A child’s alkaline electrophoresis shows two prominent bands: one in A position and one in S/D/G region. Solubility test is POSITIVE.

Why was the solubility test necessary?

Because alkaline electrophoresis cannot distinguish S from D or G.
Explanation: They co-migrate at pH 8.6.

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3.1: A 32-year-old Asian woman with lifelong anemia: RBC 6.4, Hgb 7.9, MCV 67, RDW 32.6. What is precipitating in the cells labeled a & b?

• Cell a: Hb H inclusions (β4 tetramers)
• Cell b: Heinz bodies
Explanation: Brilliant cresyl blue stain reveals different precipitates:
• Hb H → multiple “golf-ball” inclusions
• Heinz bodies → denatured hemoglobin.

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3.2: A 32-year-old Asian woman with lifelong anemia: RBC 6.4, Hgb 7.9, MCV 67, RDW 32.6.

What are the “common names” for these cells?

• Hb H cell (“golf-ball cell”)
• Bite cell (if Heinz bodies removed)
Explanation: Hb H = many evenly spaced inclusions; Heinz bodies often removed by spleen → bite cells.

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3.3: A 32-year-old Asian woman with lifelong anemia: RBC 6.4, Hgb 7.9, MCV 67, RDW 32.6.

Electrophoresis interpretation: pH 8.4 shows presence of Hb H. What hemoglobinopathy does this patient have?

Hb H Disease (α-thalassemia with three gene deletions).
Explanation:
• Very low MCV
• Normal to ↑ RBC count
• Asian ethnicity
• Hb H inclusions present
→ Classic for Hb H dis