Acute Chest Syndrome in Sickle Cell Disease: Clinical Presentation and Course

Abstract

Acute chest syndrome (ACS) is an important cause of morbidity and mortality in sickle cell disease (SCD). Previous studies reported conflicting pictures of ACS making therapeutic interventions difficult. The Cooperative Study of Sickle Cell Disease prospectively followed 3,751 patients enrolled from birth to 66 years of age for ACS. Data on presenting signs and symptoms, laboratory findings, and hospital course were collected. There were 1,722 ACS episodes in 939 patients. Young children (age 2 to 4 years) presented with fever and cough, a negative physical exam, and rarely had pain. Adults were often afebrile and complained of shortness of breath, chills, and severe pain. Upper lobe disease was more common in children; multilobe and lower lobe disease affected adults more often. Severe hypoxia occurred in 18% of adults tested and could not be predicted by examination or laboratory findings. Bacteremia was documented in 3.5% of episodes, but was strongly influenced by age (14% of infants and 1.8% of patients <10 years). ACS was most common in winter with children having the most striking increase. Transfusion was used less frequently, but earlier in children. Young children were hospitalized for 5.4 days versus 9 days for adults. Fifty percent of adults had a pain event in the 2 weeks preceding ACS and children were more likely to have febrile events. The death rate was four times higher in adults than in children. Fatal cases generally developed rapid pulmonary failure and one third were associated with bacteremia. Age has a striking effect on the clinical picture of ACS. In children, ACS was milder and more likely due to infection, whereas in adults ACS was severe, associated with pain and had a higher mortality rate.

Candida albicans induced acute chest syndrome in sickle cell disease

“…Children often have a febrile illness preceding the development of ACS and are admitted for VOC with normal chest examination and radiograph. 2,8 Nearly half of all ACS episodes occur between 1 and 3 days after admission for VOC as seen in our patient, suggesting that VOC may be a prodromal event for the development of ACS. 2 Children with concomitant asthma and sickle cell disease have increased episodes of ACS.…”

Acute chest syndrome: sickle cell disease

“…Other age groups followed a similar though less-pronounced pattern. ACS incidences are significantly higher in children aged 0-4 yr than in adults during the month of December (5).…”

“…Fourteen percent of children with SCD under 2 yr had bacteremia associated with ACS, and pneumococcus was the etiology in 78% of these cases. Children aged 0-4 yr with bacteremia had temperatures 39°C, elevated pulse rate, elevated respiration rate, upper lobe involvement, and pleural effusion more often than children without bacteremia (5). In contrast to young children, only 1.8% of patients aged 10 and older had bacteremia, and only 25% were attributable to pneumococcus.…”

“…It is important, therefore, to include chest radiography in evaluating patients with SCD who have fever, chest pain, or respiratory symptoms. Infiltrates may not appear until 2 or 3 d later (3,5,75,76), however. ACS frequently develops after admission for VOC.…”

“…With the onset of ACS, hemoglobin and white blood cell count showed a significant change from steady-state values with Hb dropping an average of 0.7 g ⁄ dL and white blood cell count increasing by 69% (5), usually accompanied by an increased reticulocyte count (5,77,81). Platelet count may be relatively decreased in some patients at the onset of ACS, but thrombocytosis is common during convalescence (5,77,81).…”