ZyraDoc #53 - September 23, 2026

A 20-year-old male with known Sickle Cell Anemia (HbSS) presents with a 12-hour history of severe, excruciating pain in his lower back, thighs, and chest following a mild upper respiratory infection.

On physical examination, he is distressed, febrile (38.2°C), tachycardic, and jaundiced, with severe bony tenderness over his femora and lumbar spine, but without focal pulmonary consolidation.

Laboratory evaluation shows severe normocytic anemia (Hb 6.8 g/dL), marked reticulocytosis (12%), sickled erythrocytes on peripheral smear, and a HbS level of 85% on hemoglobin electrophoresis, confirming acute vaso-occlusive crisis.

Case Summary

Clinical Focus:

The diagnosis is Sickle Cell Disease presenting with an Acute Vaso-Occlusive Crisis (VOC).

Aetiology/Cause:

Homozygous point mutation in the beta-globin gene (GAG → GTG), replacing glutamic acid with valine at position 6 (HbS).
Deoxygenation causes HbS polymerization, altering red blood cells into rigid, sickle-shaped forms.
Microvascular occlusion, tissue ischemia, reperfusion injury, and systemic inflammation triggered by infection, dehydration, hypoxia, cold, or stress.

Clinical Features:

Sudden onset of severe, deep, throbbing pain localized to bones, joints, back, abdomen, or chest.
Jaundice, scleral icterus, and dark urine secondary to chronic intravascular and extravascular hemolysis.
Low-grade fever and tachycardia.
History of recurrent painful crises, dactylitis (in infants), or prior hospitalizations.

Diagnosis and Investigations:

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