VASCULAR OCCLUSIVE CRISES IN SICKLE-CELL- ANEMIA

History and findings: Severe pain suddenly occurred in the shaft of th e right thigh in a 21-year-old patient of Turkish descent with known h omozygotic sickle cell anaemia. He also had marked scleral jaundice an d anaemia (haemoglobin 9.1 g/dl). An X-ray film of the lower leg point ed to a bone infarct. Type of pain, partial pressure of oxygen in bloo d (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 haem oglobin, indicated a vascular occlusive crisis in sickle cell anaemia. Treatment and course: The symptoms regressed within 8 days of the pat ient 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 pO(2) 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 1/min). His condition quickl y improved. Conclusions: in patients with sickle cell anaemia it is im portant (1) to regard crises as potentially life-threatening condition s, (2) to detect and treat infections as possible causes early and (3) to provide sufficient oxygen insufflation quickly, especially in pulm onary infections.