A 35-year-old man of African descent presents with acute hemolytic crisis triggered by infection. Laboratory findings show hemoglobin 7.5 g/dL, reticulocyte count 18%, elevated indirect bilirubin, and positive osmotic fragility test. Peripheral blood smear shows spherocytes and polychromasia. What is the drug of choice for acute hemolytic episodes in hereditary spherocytosis?

Question

Accepted Answer

A. Splenectomy. ## Clinical Diagnosis The clinical presentation is consistent with **Hereditary Spherocytosis (HS)**: - Positive osmotic fragility test (hallmark finding) - Spherocytes on blood smear - Elevated reticulocyte count and indirect bilirubin - Acute hemolytic crisis triggered by infection ## Drug of Choice: Splenectomy **Key Point:** Splenectomy is the **definitive treatment** for symptomatic hereditary spherocytosis. It removes the primary site of extravascular hemolysis and is curative in ~80–90% of cases. **High-Yield:** HS is an **extravascular hemolytic anemia** — spherocytes are rigid and trapped in the spleen's red pulp. Removing the spleen eliminates the hemolytic mechanism. **Clinical Pearl:** Splenectomy is indicated in: 1. Moderate to severe hemolysis (Hb < 10 g/dL, reticulocyte count > 10%) 2. Recurrent hemolytic crises 3. Symptomatic patients (jaundice, gallstones, growth retardation in children) ## Pathophysiology of HS ```mermaid flowchart TD A[Defect in RBC membrane proteins
Spectrin, ankyrin, band 3]:::outcome --> B[Loss of membrane stability] B --> C[RBC becomes spherical
Less deformable] C --> D[Trapped in splenic red pulp] D --> E[Extravascular hemolysis]:::urgent F[Splenectomy]:::action --> G[Removes hemolytic organ] G --> H[Hemolysis resolves]:::outcome ``` ## Management Strategy in HS | Phase | Intervention | Rationale | |-------|-------------|----------| | **Acute crisis** | Transfusion, hydration, folic acid | Supportive; manage complications | | **Between crises** | Folic acid supplementation | Prevent folate depletion from chronic hemolysis | | **Definitive** | **Splenectomy** | **Removes site of hemolysis** | | **Post-splenectomy** | Pneumococcal, meningococcal, H. influenzae vaccination | Prevent overwhelming sepsis | **Mnemonic:** SHIV (Splenectomy, Hemolytic anemia, Infection prophylaxis, Vaccination) — remember to vaccinate before splenectomy. **Warning:** Do NOT confuse HS (extravascular) with PNH (intravascular). Splenectomy works in HS but not PNH.

Answer

B. Corticosteroids

Answer

C. Folic acid supplementation

Answer

D. Penicillin prophylaxis

Explanation

Clinical Diagnosis

Drug of Choice: Splenectomy

Pathophysiology of HS

Management Strategy in HS

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Phase	Intervention	Rationale
Acute crisis	Transfusion, hydration, folic acid	Supportive; manage complications
Between crises	Folic acid supplementation	Prevent folate depletion from chronic hemolysis
Definitive	Splenectomy	Removes site of hemolysis
Post-splenectomy	Pneumococcal, meningococcal, H. influenzae vaccination	Prevent overwhelming sepsis