[Severe upper abdominal pain during a long distance flight].

HISTORY AND CLINICAL FINDINGS: A 43-year-old woman of Ghanaian origin presented with severe upper abdominal pain starting on a long distance flight. Physical examination revealed tenderness on palpation in the left upper abdomen and flank. There was no report of pre-existing conditions or permanent medication in the medical history.

INVESTIGATIONS AND DIAGNOSIS: Laboratory tests showed signs of haemolytic anemia and elevated inflammatory parameters. The "thick blood smear" was normal. Ultrasonography revealed an enlarged spleen (14×5 cm) with inhomogeneous parenchyma and vast, diffusely spread hypoechoic lesions in perihilar location, interpreted as extended splenic infarction. Symptom onset on a long distance flight, haemolytic anemia and extended splenic infarction led to the assumption of a vasoocclusive crisis with haemolysis. Moleculargenetic tests proved the presence of HbSC-sickle cell disease and heterozygous alpha-thalassemia.

TREATMENT AND COURSE: After infusion of crystalloid solution the patient was asymptomatic further on. Due to splenic infarction she received prophylactic treatment with Cefuroxim. A vaccination against pneumococci, meningococci and Haemophilus influenza B was recommended.

CONCLUSIONS

Mild hypoxia and dehydration on a long distance flight can trigger a sickle cell crisis and may contribute to late clinical manifestation and diagnosis of sickle cell disease in some cases. Patients suffering from HbSC-sickle cell disease are at risk for the same life-threatening complications as patients with HbSS-sickle cell disease. HbSC-sickle cell disease should not be considered as a mild form of HbSS-sickle cell disease but as a separate disease with specific clinical manifestations. In contrast-enhanced ultrasonography, splenic infarction due to sickle cell crisis may markedly differ from "typical" arterial thromboembolic infarction.