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Pulmonary fat embolism: a distinct cause of severe acute chest syndrome in sickle cell anemia
Open accessAbstract
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.
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Fat and marrow emboli lodge in the pulmonary microcirculation, resulting in infarction.
“fat and marrow emboli lodge in the pulmonary microcirculation, resulting in infarction (212).”
From Pathophysiology of Sickle Cell Disease (2019) · cites this paper as [212]
The Discussion (page unavailable) describes fat emboli in the lungs and vascular obstruction with injury to the pulmonary microvasculature. It does not say that fat and marrow emboli cause pulmonary infarction; the infarction it describes occurs in bone before fat embolization. (LLM, conf=0.84)
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References 50 · Cited by 261
Metadata
- Authors
- Vichinsky, E., Williams, R., Das, M., Earles, A., Lewis, N., Adler, A., & McQuitty, J.
- Affiliations
- Department of Hematology/Oncology, Children's Hospital Oakland, CA 94609.
- ORCID
- Vichinsky, E
- Venue
- Blood
- Publisher
- American Society of Hematology
- Published
- 1994-06-01
- Volume
- 83
- Issue
- 11
- Pages
- 3107-3112
- ISSN
- 0006-4971 (ISSN-L)
- 1528-0020
- OpenAlex
- W194543173
- Cited by
- 261
- References
- 50
- Citations by year
- 2026: 1 · 2025: 7 · 2024: 4 · 2023: 23 · 2022: 10 · 2021: 8 · 2020: 2 · 2019: 6 · 2018: 4 · 2017: 5 · 2016: 3 · 2015: 11 · 2014: 8 · 2013: 8 · 2012: 6
- FWCI
- 6.17
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Blood (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institutes of Health (5p60hl020985-25)
- National Heart, Lung, and Blood Institute (HL20985)
- Topics
- Health Sciences › Medicine › Genetics (clinical) › Hemoglobinopathies and Related Disorders
- Health Sciences › Medicine › Internal Medicine › Venous Thromboembolism Diagnosis and Management
- Health Sciences › Medicine › Hematology › Methemoglobinemia and Tumor Lysis Syndrome
- Keywords
- pulmonary fat embolism, acute chest syndrome, sickle cell disease, bronchoalveolar lavage, lung macrophages, erythroblasts, platelet count, hemoglobin decrease, bone pain, chest pain, neurological symptoms, pediatric patients, differential diagnosis
- MeSH terms
- Acute Disease, Adolescent, Adult, Anemia, Sickle Cell, Bronchoalveolar Lavage Fluid, Bronchoscopy, Child, Child, Preschool, Embolism, Fat, Humans, Infant, Lung Diseases
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