[Severe upper abdominal pain during a long distance flight]

Area of Science:

• Hematology
• Genetics

Background:

• Sickle cell disease (SCD) encompasses various genotypes, including HbSC-sickle cell disease.
• HbSC-SCD is often perceived as milder than HbSS-SCD, but can present with severe complications.

Purpose of the Study:

• To highlight the potential for environmental triggers, like long-distance travel, to precipitate vaso-occlusive crises in HbSC-SCD.
• To emphasize that HbSC-SCD is a distinct entity with specific clinical manifestations and risks, not a mild variant of HbSS-SCD.

Main Methods:

• Case report of a 43-year-old woman presenting with severe abdominal pain.
• Diagnostic workup included laboratory tests (hemolysis, inflammation), ultrasonography (splenic infarction), and molecular genetic testing (HbSC-SCD, alpha-thalassemia).

Main Results:

• The patient experienced a vaso-occlusive crisis triggered by a long flight, presenting with hemolytic anemia and splenic infarction.
• Molecular testing confirmed HbSC-SCD with heterozygous alpha-thalassemia.
• Ultrasonography revealed extensive splenic infarction, differing from typical arterial thromboembolic infarction.

Conclusions:

• Environmental factors like hypoxia and dehydration during travel can precipitate sickle cell crises in HbSC-SCD.
• HbSC-SCD carries risks comparable to HbSS-SCD and should be recognized as a separate disease entity.
• Splenic infarction in sickle cell crisis may have distinct ultrasonographic features.