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[Sickle cell trait: clinical presentation as acute liver failure]
A H Ferrari1, T Miyagui, F R Rodrigues
1Departamento de Patologia, Universidade Federal Fluminense (UFF), Hospital Universitário Antônio Pedro (HUAP).
Arquivos De Gastroenterologia
|August 26, 1998
Summary
Sickle cell trait can cause acute liver failure and ischemic damage in multiple organs, even without a diagnosis of sickle cell disease. This case highlights the potential for severe complications, including heart disease and pneumopathy, in individuals with sickle cell trait.
Area of Science:
- Hematology
- Pathology
- Internal Medicine
Background:
- Sickle cell trait is often considered benign, but its potential to cause significant pathology is increasingly recognized.
- This case examines a fatal presentation involving multiple organ systems in an adult male with undiagnosed sickle cell trait.
Observation:
- A 36-year-old male presented with acute liver insufficiency, heart disease, pneumopathy, and infectious syndrome.
- Necropsy revealed anatomopathological changes consistent with sickle cell trait, including acute ischemic lesions in the heart, kidneys, central nervous system, and liver.
Findings:
- Ischemic lesions were attributed to circulatory disturbances secondary to sickle cell trait, particularly sinusoid occlusion and hepatocyte necrosis leading to hepatic failure.
- Respiratory insufficiency from lung disease may have triggered sickling episodes, causing recent ischemic damage.
- Chronic ischemic heart lesions suggest prior sickling events.
Implications:
- Sickle cell trait should be considered in the differential diagnosis of unexplained ischemic organ damage, even in the absence of overt anemia.
- This case underscores the importance of recognizing sickle cell trait as a potential risk factor for serious medical conditions.
- Family screening identified another individual with sickle cell trait, emphasizing the hereditary nature of the condition.