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Predictors of Impending Acute Chest Syndrome in Patients with Sickle Cell

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Predictors of Impending Acute Chest Syndrome in Patients with Sickle Cell www.nature.com/scientificreports OPEN Predictors of impending acute chest syndrome in patients with sickle cell anaemia Salam Alkindi1,2*, Ikhlas Al-Busaidi1, Bushra Al-Salami1, Samir Raniga3, Anil Pathare 1 & Samir K. Ballas4 Acute chest syndrome (ACS) is a major complication of sickle cell anaemia (SCA) and a leading cause for hospital admissions and death. We aimed to study the spectrum of clinical and laboratory features of ACS and to assess the predisposing factors and predictors of severity. A retrospective case-control cohort was studied by retrieving patient information from electronic medical records after ethical approval. One hundred adolescents and adults with SCA and hospital admissions for ACS were identifed through the discharge summaries, along with 20 additional patients presenting with VOC, but without ACS (controls). Among the patients with ACS, fever (>38.5 °C), reduced oxygen saturation (<95) and asplenia signifcantly difered when compared to those of controls (p < 0.05, chi-squared test). The degree of severity was refected in the use of non-invasive ventilation (NIV), simple and exchange transfusions, and the presence of bilateral pleural efusions and multi-lobar atelectasis/consolidation, which were signifcantly higher in the cases with ACS than in the controls. Lower haemoglobin (Hb) and high WBC counts were also signifcantly diferent between the two groups (p < 0.05, Student’s t test). Using logistic regression, our study further demonstrated that asplenia, fever, and reduced O2 saturation, along with low Hb and leukocytosis, were important predictors for the development of ACS. Sickle cell anaemia (SCA) is an autosomal recessive disorder characterized by a point mutation in codon 6 of the beta globin chain, where glutamic acid is replaced by valine, resulting in the formation of HbS with varied clinical 1 manifestations . Te abnormal Hb is insoluble and polymerizes when exposed to low O2 tension, leading to the major clinical manifestations of SCA, including recurrent vaso-occlusive crisis (VOC), predisposition to infec- tions and reduced red cell survival with anaemia2. Sickle cell disease (SCD) is prevalent in sub-Saharan Africa and the Indian sub-continent, but human pop- ulation mobility has spread the disorder throughout Europe, Asia and the Americas3,4. SCD is highly prevalent in Oman, with an estimated sickle cell gene frequency of 5.8% and a prevalence of sickle cell traits and SCA in Omani children of 4.8% and 0.3%, respectively5,6. It is thus estimated that one neonate of every 323 newborns in Oman has SCA6. Acute chest syndrome (ACS) is defned as the presence of fever and/or new respiratory symptoms accom- panied by the presence of a new pulmonary infltrate on chest X-ray and is one of the most important causes of death and the second most common cause of hospitalization in SCA patients7. In a retrospective study, ACS was found to be responsible for 69.6% of SCA ICU admissions and was associated with a 16% mortality rate7. Furthermore, ACS is associated with a high risk of sickle cell-related mortality and morbidity, including pro- longed hospitalization8. Although the aetiology of ACS is multifactorial, the Cooperative Study of Sickle Cell Disease (CSSCD) in the United States identifed several aetiological factors, including infections (bacterial, viral and others), thrombosis, fat and pulmonary embolism, but in 50% of the cases, a clear aetiology could not be identifed9. Furthermore, in the same study, high Hb, low HbF and high WBC counts during steady state were identifed as risk factors for the development of ACS. It is also well known that many patients with SCA develop ACS afer being admitted to the hospital9. Styles et al.10 observed that secretory phospholipase A2 (sPLA2) demonstrated a 67% specifcity with 100% sensitivity to predict impending ACS in the patients they studied. Tis observation was further substantiated by Ballas et al.11 in 80% of SCA patients in their study. However, Styles et al., 1Department of Haematology, Sultan Qaboos University Hospital, Muscat, Oman. 2College of Medicine & Health Sciences, Muscat, Oman. 3Department of Radiology & Molecular Imaging, Sultan Qaboos University Hospital, Muscat, Oman. 4Cardeza Foundation for Hematologic Research, Thomas Jeferson University, Philadelphia, USA. *email: [email protected] SCIENTIFIC REPORTS | (2020) 10:2470 | https://doi.org/10.1038/s41598-020-59258-y 1 www.nature.com/scientificreports/ www.nature.com/scientificreports in a more robust randomized study design, demonstrated a 71% specifcity with 73% sensitivity, although the pos- itive predictive value of sPLA2 was only 24%12. Unfortunately, the test is not yet available commercially. Terefore, the aim of this study was to evaluate the clinical and laboratory features of patients with ACS compared to patients with simple VOC and to assess the risk factors predisposing the development of ACS and potential predictors of its severity. Materials and Methods Tis is a single institution case-control cohort study of SCA patients, who were admitted with ACS between July 2009 and June 2017 at our institution. A protocol was submitted to the Medical Research and Ethics Committee (MREC) to collect relevant details of consecutive cases from the medical records of patients who were admitted for ACS. All patients were Omani Arabs, above 15 years of age, with SCA (HbSS and HbSβ°), whereas other SCD subtypes, such as SD, SC, and SE, were excluded. Ninety-two SCA patients (100 episodes) were identifed through the discharge summaries as having had ACS, and 20 SCA patients with simple VOC (20 episodes) who did not have ACS were used as the controls during the same time frame. Te controls had an acute episode of severe pain in spite of the administration of analgesic medication and needed admission without any respiratory symptoms. Tese controls were selected consecutively in a 1:5 proportion during the same time frame as the enrolment of cases. Acute chest syndrome was defned as patients presenting with fever and/or respiratory symptoms, accom- panied by new infltrates on the chest radiograph. All methods were carried out in accordance with relevant local guidelines and regulations. Te MREC approval (MREC #1333) was for a retrospective data collection and anal- ysis; hence, individual patient consent was not required/mandated by the MREC. Te study evaluated clinical and laboratory parameters, including the vital signs such as heart rate, blood pressure, respiratory rate and pulse oximetry O2 saturation, for both groups of patients throughout the whole period of admission. Other clinical parameters that were studied included fever, chest/back pain, cough, wheez- ing, crepitation and use of non-invasive ventilation (NIV). Te laboratory parameters included Hb, HbF, retic- ulocyte count, WBC count, platelet count, serum LDH, AST, ALT, serum bilirubin, serum creatinine, and CRP. Blood culture studies and the use of hydroxyurea (HU) and simple and exchange transfusion were also recorded. Radiological investigations recorded fndings using chest X-rays, abdominal ultrasounds and chest CT scans. Statistical analysis. Te Statistical Package for Social Science (IBM SPSS, USA ver.23) was used to analyse the collected data. Normally distributed data were characterized as means with standard deviations, whereas data that were skewed [i.e., not normally distributed] were characterized as medians with interquartile ranges (IQRs) for continuous variables and as percentages and frequencies for categorical variables. Te chi-squared test was used to study the statistical signifcance of qualitative variables, whereas Student’s t test was used to study the diferences in the quantitative parameters between the cases and controls. Logistic regression with odds ratios and 95% confdence intervals (CIs) was performed to ascertain the efectiveness of the predictors of ACS. A p-value < 0.05 was considered statistically signifcant. Results Although there was a similar frequency of males in the cases and controls, males tended to sufer from ACS at an earlier median age than females (27 years compared to 31 years in females), but this was not statistically signifcant. History of previous ACS and hydroxyurea therapy was also similar between the two groups (Table 1). Fever, crepitation and reduced O2 saturation by pulse oximetry were signifcantly abnormal in the cases with ACS (p < 0.05, chi-squared test). Tis was also refected in the signifcantly higher odds ratios in the cases com- pared to the controls (p < 0.05). Radiologically, abdominal ultrasound demonstrated a signifcantly higher degree of asplenia in the cases than in the controls (p < 0.05, chi-squared test). Pleural efusions, positive chest X-rays and CT scans of the chest as well as multilobar atelectasis were signifcantly greater in the cases than in the control (p < 0.05, chi- squared test). Complete blood counts showed that Hb nadirs and maximal WBC counts showed a statistically signifcant diference between the two groups (p < 0.05, Student’s t test) with logistic regression analysis also confrming the signifcance (Table 2). HbF levels did not show any signifcant diferences between the cases and controls. Furthermore, among the biochemical parameters, although serum CRP, serum total bilirubin, and serum LDH levels were elevated, they did not reach statistical signifcance (Table 1). Te blood cultures were positive in 4% of the cases compared to none in the controls. Furthermore, the use of NIV was reported in 16.3% of the cases compared to none in the controls. Sixty-three percent of patients in the case group received simple blood transfusions compared to 20% in the control group, whereas exchange transfusions were given in 66.3% of ACS patients compared to 10% of the controls (p < 0.05, chi-squared test). Te transfusions were based on the clinical evaluations and therapeutic decisions of the treating physicians. Only one death was recorded in the ACS patients compared to none in the control group. Discussion SCA is the most prevalent hereditary haemoglobin disorder in Oman5,6,12,13.
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