J. Riehl et al., ACUTE CHEST SYNDROME AND CAVITARY PULMONA RY TUBERCULOSIS WITH SICKLE-CELL DISEASE, Deutsche Medizinische Wochenschrift, 121(44), 1996, pp. 1354-1358
History and clinical findings: A 17-year-old girl from Zaire was admit
ted to hospital with fever, cough, dyspnoea and severe chest pain. In
addition to marked anaemia (haemoglobin 6.6 g/dl) she was known to hav
e cavitary/exudative pulmonary tuberculosis (PTb) with bilateral basal
infiltrations. Investigations: Blood gas analysis indicated partial r
espiratory failure (pO(2) 55 mm Hg, pCO(2) 36 mm Hg). Blood smear unde
r air exclusion showed erythrocyte sickling. Haemoglobin electrophores
is demonstrated 92.7% HbS and thus confirmed sickle cell anaemia. A sm
all spleen on sonography and the presence of Howell-lolly bodies were
interpreted as signs of functional asplenia. Microbiological and radio
logical tests confirmed exudative-cavitary PTb. Treatment and course T
he findings were interpreted as due to an acute chest syndrome, caused
by sickle cell thrombi in the pulmonary brood vessels, precipitated b
y the PTb. Transfusion of two units of erythrocyte concentrates led to
improvement of the chest pain and the respiratory failure within a fe
w hours. The PTb was successfully treated without any complications. C
onclusion: Acute chest syndrome is a vascular occlusive complication o
f sickle cell disease, pulmonary tuberculosis precipitating the develo
pment of this acute condition. Administration of erythrocyte concentra
te rapidly improves the signs and symptoms.