Golden Tracheal Secretions and Bronchoalveolar Fluid During Acute Chest Syndrome in Sickle Cell Disease ======================================================================================================= * Damien Contou * Armand Mekontso Dessap * Guillaume Carteaux * Christian Brun-Buisson * Bernard Maitre * Nicolas de Prost ## Abstract Acute chest syndrome (ACS) is the leading cause of ICU admission in patients with sickle cell disease and is characterized by golden sputum, which is commonly attributed to the presence of bilirubin. Three young consecutive patients with homozygous sickle cell disease were admitted for severe acute respiratory syndrome due to ACS. In all 3 patients, tracheal secretions and bronchoalveolar lavage fluid (BALF) showed a yellowish plasma-like stain. After normalization for the plasma-to-BAL urea ratio, BALF protein and lactate dehydrogenase levels were consistent with an exudative process. BALF bilirubin concentrations were very low, implying that the yellowish stain was not related to bilirubin content. The yellowish coloration of tracheal secretions and BALF observed during ACS appears to be related to an intense exudative process rather than to the presence of bilirubin. * sickle cell disease * acute chest syndrome * yellow sputum * acute respiratory distress syndrome * pulmonary fat embolism * intensive care ## Introduction Acute chest syndrome (ACS) is the leading cause of ICU admission in patients with sickle cell disease.1 The main complication of ACS is severe acute respiratory syndrome, which can lead to refractory hypoxemia and death. Golden sputum is a hallmark of ACS and is commonly attributed to the presence of bilirubin, also termed bilioptysis,2 even though this term initially referred to the existence of a bronchobiliary fistula. ## Case Reports Three young female patients with known homozygous sickle cell disease were admitted to our ICU over a 1-week period for acute respiratory failure due to ACS following painful vaso-occlusive crises. The baseline characteristics of sickle cell disease, clinical and laboratory features upon ICU admission, and outcomes of these 3 patients are reported in Table 1. All 3 developed severe acute respiratory syndrome, leading to tracheal intubation, prone positioning, and nitric oxide inhalation treatment. Intravenous antibiotic treatment, combining a third-generation cephalosporin and a macrolide, was administered to all 3 patients, but bacterial blood cultures and respiratory samples remained sterile. The condition improved in 2 patients, who were eventually discharged from the ICU 2 weeks later (patients 1 and 3), whereas one patient (patient 2) required venovenous extracorporeal membrane oxygenation and died on day 5. View this table: [Table 1.](http://rc.rcjournal.com/content/60/4/e73/T1) Table 1. Presentation and Outcomes of Patients With Severe Acute Chest Syndrome In all 3 patients, tracheal secretions and bronchoalveolar lavage fluid (BALF) showed a yellowish plasma-like stain (Fig. 1, B–D; see the supplementary video at [http://www.rcjournal.com](http://www.rcjournal.com)). After normalization for the plasma-to-BAL urea ratio, BALF (vs plasma) protein (patient 1, 54 vs 57 g/L; patient 2, 45 vs 55 g/L) and lactate dehydrogenase (patient 1, 1,890 vs 1,529 U/L; patient 2, 795 vs 512 U/L) levels were similar and consistent with an exudative process. BALF bilirubin concentrations were 7 (patient 1) and 5 (patient 2) μmol/L, implying that the yellowish stain was not related to bilirubin content. Interestingly, tracheal secretions of patient 3 exhibited the same color as her plasma (Fig. 1C). Finally, in patients 1 and 2, 30% of alveolar macrophages were stained with Oil Red O (Fig. 1E), indicative of lung fat embolism.3–5 ![Figure 1.](http://rc.rcjournal.com/https://rc.rcjournal.com/content/respcare/60/4/e73/F1.medium.gif) [Figure 1.](http://rc.rcjournal.com/content/60/4/e73/F1) Figure 1. Golden tracheal aspirates and bronchoalveolar lavage fluid (BALF) in patients with severe acute chest syndrome. A: Contrast-enhanced chest computed tomography scan depicting typical bilateral lung base consolidations in patient 1. B: Tracheal secretions from patient 1. C: Tracheal secretions (left tube) and plasma (right tube) from patient 3 showing the same yellowish color. D: Macroscopically golden BALF from patient 1. E: Cytological examination of BALF from patient 1 showing alveolar macrophages stained with Oil Red O. ## Discussion Golden sputum is a classic and pathognomonic sign of ACS, which is often associated with pulmonary fat embolism.3–5 This yellowish coloration appears to be related to an intense exudative process rather than to the presence of bilirubin. Of course, this finding has limited clinical implications for the current management of patients with ACS. However, understanding the pathophysiologic phenomena underlying this yellowish stain, which is not commonly reported during ARDS in patients without sickle cell disease, could be of paramount importance for the management of ACS. Future studies aiming at determining the components of this peculiar alveolar fluid (eg, using proteomics) could allow for the identification of specific diagnostic or therapeutic markers of ACS. ## Acknowledgment We thank Dr Jeanne Tran Van Nhieu for providing images of macrophages stained with Oil Red O. ## Footnotes * Correspondence: Nicolas de Prost MD PhD, Service de Réanimation Médicale, Groupe Henri Mondor-Albert Chenevier, Assistance Publique Hôpitaux de Paris, 51 avenue du Maréchal de Lattre de Tassigny, 94010 Créteil Cedex, France. E-mail: nicolas.de-prost{at}hmn.aphp.fr. * Supplementary material related to this paper is available at [http://www.rcjournal.com](http://www.rcjournal.com). * The authors have disclosed no conflicts of interest. * Copyright © 2015 by Daedalus Enterprises ## References 1. 1. Cecchini J, Lionnet F, Djibré M, Parrot A, Stojanovic KS, Girot R, Fartoukh M. Outcomes of adult patients with sickle-cell disease admitted to the ICU: a case series. 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