[Vaso-occlusive crises in sickle-cell anemia]. / Vaso-okklusive Krisen bei Sichelzellanämie.

ABSTRACT

HISTORY AND FINDINGS: Severe pain suddenly occurred in the shaft of the right thigh in a 21-year-old patient of Turkish descent with known homozygotic sickle cell anaemia. He also had marked scleral jaundice and anaemia (haemoglobin 9.1 g/dl). An X-ray film of the lower leg pointed to a bone infarct. Type of pain, partial pressure of oxygen in blood (70 mm Hg), concentration of lactate dehydrogenase and the bilirubin level (4,5 mg/dl) as signs of haemolysis, as well as the fall in haemoglobin, indicated a vascular occlusive crisis in sickle cell anaemia. TREATMENT AND COURSE The symptoms regressed within 8 days of the patient receiving fluids, analgesics and oxygenation by nasal tube. A few weeks later he developed a fever, productive cough and severe pain in joints and abdomen. Blood p02 was 54 mm Hg. Pneumonia in the right lung base was the cause of this renewed life-threatening crisis, which was again associated with haemolysis. Klebsiella having been found in sputum he was treated with cefotaxim (1 g twice daily intravenously) and oxygen insufflation via nasal tube (7 l/min). His condition quickly improved. CONCLUSIONS: In patients with sickle cell anaemia it is important (1) to regard crises as potentially life-threatening conditions, (2) to detect and treat infections as possible causes early and (3) to provide sufficient oxygen insufflation quickly, especially in pulmonary infections.