Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening inflammatory syndrome that may complicate hematologic malignancies (HM). The appropriateness of current criteria for diagnosing HLH in the context of HMs is unknown because they were developed for children with familial HLH (HLH-2004) or derived from adult patient cohorts in which HMs were underrepresented (HScore). Moreover, many features of these criteria may directly reflect the underlying HM rather than an abnormal inflammatory state. To improve and potentially simplify HLH diagnosis in patients with HMs, we studied an international cohort of 225 adult patients with various HMs both with and without HLH and for whom HLH-2004 criteria were available. We used classification and regression tree and receiver operating curve analysis to identify the most useful diagnostic and prognostic parameters and optimize laboratory cutoff values. Combined elevation of soluble CD25 (>3,900 U/ml) and ferritin (>1,000 ng/ml) best identified HLH-2004 defining features (sensitivity 84%, specificity 81%). Moreover, this combination, which we term the 'optimized HLH inflammatory' (OHI) index, was highly predictive of mortality (hazard ratio 4.3; confidence interval 3.0-6.2) across diverse HMs. Furthermore, the OHI index identified a large group of patients with high mortality risk that were not defined as having HLH by HLH-2004/HScore. Finally, the OHI demonstrates diagnostic and prognostic value when used for routine surveillance of patients with newly diagnosed HMs as well as those with clinically suspected HLH. Thus, we conclude that the OHI index identifies HM patients with an inflammatory state associated with a high mortality risk and warrants further prospective validation.
<b><i>Background:</i></b> Sarcoidosis is a heterogeneous multisystemic disorder of unknown etiology. Dyspnea and fatigue are two of the most common and debilitating symptoms experienced by subjects with sarcoidosis. There is limited evidence regarding the short- and long-term impact of pulmonary rehabilitation (PR) on exercise capacity and fatigue in these individuals. <b><i>Objective:</i></b> To evaluate the benefit of PR in subjects with pulmonary sarcoidosis at different severity stages and to review the current literature about PR in sarcoidosis. <b><i>Methods:</i></b> PR included a 12-week training program of a twice-weekly 90-min workouts. Fifty-two subjects with stable pulmonary sarcoidosis were recruited. Maximal exercise capacity, defined as VO<sub>2</sub>max, was measured using the cardiopulmonary exercise test (CPET). Pulmonary function tests, 6-min walking distance (6MWD), St. George’s Respiratory Questionnaire (SGRQ), and the modified Medical Research Council (mMRC) and Hospital Anxiety and Depression Scale (HADS) questionnaires were given before and after PR and following 6 months (follow-up). <b><i>Results:</i></b> The PR program significantly increased the VO<sub>2</sub>max (1.8 ± 2.3 mL/kg/min, <i>p</i> = 0.002), following 12 weeks. mMRC and SGRQ scores were also improved (−0.3 ± 0.8, <i>p</i> = 0.03, and −3.87 ± 10.4, <i>p</i> = 0.03, respectively). The impact of PR on VO<sub>2</sub>max was more pronounced in subjects with pulmonary parenchymal involvement. The increase in VO<sub>2</sub>max correlated with initial disease severity (indicated by FEV1/FVC, <i>p</i> = 0.01). Subjects with FEV1/FVC <70% showed greater improvement in 6MWD. 6MWD also improved in those with a transfer coefficient of the lung for CO (KCO) above 80% predicted (<i>p</i> < 0.05). At 6-month follow-up, the VO<sub>2</sub>max, 6MWD, and SGRQ scores remained stable, thus suggesting lasting effects of PR. <b><i>Conclusion:</i></b> PR is a promising complementary therapeutic intervention for subjects with sarcoidosis. Further study is needed to validate these findings.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
customersupport@researchsolutions.com
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
This site is protected by reCAPTCHA and the Google Privacy Policy and Terms of Service apply.
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.