Chest
Volume 117, Issue 5, May 2000, Pages 1386-1392
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Clinical Investigations
DIFFUSE LUNG DISEASE
Acute Chest Syndrome in Adults With Sickle Cell Disease

https://doi.org/10.1378/chest.117.5.1386Get rights and content

Study objectives

Acute chest syndrome (ACS) is a frequent and potentially severe pulmonary illness in sickle cell disease (SCD). The aim of the study was to report the clinical features and outcome of consecutive ACS episodes in adult patients in a French SCD center. All patients were treated according to an uniform therapeutic protocol applying transfusion only in the more severe clinical form of ACS.

Results

There were 107 consecutive episodes in 77 adult patients (mean age, 29 ± 7 years; 78% hemoglobin [Hb] SS; 14% Hb SC; and 8% Hb Sβ + thalassemia) over a 6-year period. Seventy-eight percent of our patients had an associated vaso-occlusive crisis that preceded the chest signs in half of the cases. Comparison between acute and baseline levels showed a statistically significant difference in Hb levels (drop of 1.6 to 2.25 g/dL depending on Hb genotype), WBC count (increase of 9.2 ± 8.3 × 109/L); platelet count (increase of 67 ± 209 × 109/L); and lactate dehydrogenase values (increase of 358 ± 775 IU/L) in ACS patients. Hypercapnia was detected in 42% of patients without sign of narcotic abuse. We identified a high percentage of alveolar macrophages containing fat droplets in 31 of 43 (77%) patients who underwent BAL. Bacterial culture findings were almost always negative, but were performed after starting antibiotic therapy that was administered in 96 episodes. Transfusion was required in 50 of 107 ACS events (47%). Five patients died, and all were transfused.

Conclusions

These results confirm that fat embolism is probably a frequent mechanism of ACS in adult patients. However, fat embolism was not associated with a more severe clinical course, suggesting that bronchoscopy and BAL have little impact on the management of these patients. Restricting transfusion to the most severe ACS cases does not seem to increase the mortality rate.

Section snippets

Patients

All patients were recruited from the Sickle Cell Disease Center at the Henri Mondor University Hospital, Créteil, France, which follows a cohort of 800 adult patients. All consecutive ACS episodes in this cohort were recorded from 1991 to 1997. Hemoglobin (Hb) phenotype was determined by standard procedure to be homozygous Hb SS, Hb SC, or Hb Sβ-thalassemia. β-globin cluster haplotypes were determined as previously described.20 The diagnosis of ACS was based on the presence of fever or chest

Results

One hundred seven consecutive episodes of ACS were considered in 77 patients. Of these patients, 52 had one ACS episode, 17 had two episodes, and 7 had three episodes. For 14 patients (20%), ACS was the first severe acute event related to SCD. Table 1 shows the baseline characteristics of the 77 patients before the first episode. The haplotype distribution of patients presenting ACS was similar to that of the total patient population followed at our center. Only three patients received chronic

Discussion

The aim of this study was to determine the characteristics and outcome of ACS in adult patients. Recent studies, particularly the report of the Cooperative Study of Sickle Cell Disease (CSSCD), have described ACS clinical features in large numbers of patients.5, 1415, 1617, 1819 However, most of these studies have focused on patients < 16 years old. In contrast, our monocentric study included only adult patients treated according to an uniform protocol in which transfusion was used only for the

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