Acute chest syndrome and cavitary pulmonary tuberculosis with sickle cell disease

History and clinical findings: A 17-year-old girl from Zaire was admitted to hospital with fever, cough, dyspnoea and severe chest pain. In addition to marked anaemia (haemoglobin 6.6 g/dl) she was known to have cavitary/exudative pulmonary tuberculosis (PTb) with bilateral basal infiltrations. Investigations: Blood gas analysis indicated partial respiratory failure (pO(2) 55 mm Hg, pCO(2) 36 mm Hg). Blood smear under air exclusion showed erythrocyte sickling. Haemoglobin electrophoresis demonstrated 92.7% HbS and thus confirmed sickle cell anaemia. A small spleen on sonography and the presence of Howell-lolly bodies were interpreted as signs of functional asplenia. Microbiological and radiological tests confirmed exudative-cavitary PTb. Treatment and course The findings were interpreted as due to an acute chest syndrome, caused by sickle cell thrombi in the pulmonary brood vessels, precipitated by the PTb. Transfusion of two units of erythrocyte concentrates led to improvement of the chest pain and the respiratory failure within a few hours. The PTb was successfully treated without any complications. Conclusion: Acute chest syndrome is a vascular occlusive complication of sickle cell disease, pulmonary tuberculosis precipitating the development of this acute condition. Administration of erythrocyte concentrate rapidly improves the signs and symptoms.