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Next Article 
Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in
sickle cell anemia
E Vichinsky, R Williams, M Das, AN Earles, N Lewis, A Adler and J McQuitty
Department of Hematology/Oncology, Children's Hospital Oakland, CA 94609.
The etiology of most cases of acute chest syndrome (ACS) in sickle cell
disease (SCD) is unknown. Although pulmonary fat embolism (PFE) is
frequently found on autopsy, it is rarely considered in the differential
diagnosis in pediatric patients. We conducted a study to determine if we
could identify PFE in SCD patients with ACS, define the clinical and
laboratory course of PFE, and determine if bronchoalveolar lavage is safe
and useful in diagnosis of PFE. Twenty-seven SCD patients with ACS were
evaluated and compared with 43 control patients. Serial tests (complete
blood count, platelet count, nucleated red blood cells [NRBCs], chest
x-ray, and oxygen saturations) were compared with steady-state results.
Diagnosis of PFE was made by quantitative evaluation of pulmonary
macrophages for intracellular fat. No serious complications from
bronchoscopy were observed. In the SCD patients with ACS, 12 were PFE+ and
15 were PFE-. The clinical course of the two groups was quite different.
All PFE+ patients experienced bone pain and 11 of 12 had chest pain. In
contrast, only 6 of 15 of PFE- patients had bone or chest pain. Neurologic
symptoms developed in 6 of 12 of the PFE+ group and in none of the PFE-
group. Mean hospital days for PFE+ was 13 compared with 7 for PFE-.
Laboratory studies in PFE+ showed a significant decrease in hemoglobin (-2
g, P < .05), platelet count (- 293,000, P < .001), and an increase in
NRBCs/100 white blood cells (+8.3, P < .001) compared with PFE-. These
results indicate that when PFE is associated with ACS, it is characterized
by a distinct clinical course, and that bronchial lavage is a safe and
useful test in diagnosing PFE in patients with ACS.
Volume 83,
Issue 11,
pp. 3107-3112,
06/01/1994
Copyright © 1994 by The American Society of Hematology
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