Increased platelet activation occurs in cystic fibrosis patients and correlates to clinical status

Lindberg, Ulrika; Svensson, Lisbeth; Hellmark, Thomas; Segelmark, Mårten; Shannon, Oonagh

doi:10.1016/j.thromres.2017.12.012

Cited by 24 publications

(16 citation statements)

References 41 publications

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“…Detailed clinical and laboratory features have been reported for four patients who all exhibited hypergammaglobulinemia, potentially contributing to IC generation and deposition (8). One patient exhibited thrombocytosis (509.000/ml), supporting the aforementioned hypothesis that platelets play a role in the development and persistence of inflammation, and therefore the development of vasculitis in CF (8,57). The reported extent and clinical course of vasculitis in CF is variable.…”

Section: Clinical Considerations and Treatment Optionssupporting

confidence: 57%

“…Although, in pwCF, thrombocytes aggregate more easily (potentially also as a result of aforementioned increased NETosis) and have increased capacity to form complexes with monocytes compared with healthy individuals, they produce reduced amounts of LXA 4 and fail to contribute to the resolution of inflammation (57). This process may enhance the vicious cycle that causes chronic inflammation in CF and potentially contributes to the development of vasculitis (Figure 5).…”

Section: Inability To Suppress Neutrophil Activity In Pwcfmentioning

confidence: 99%

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Vasculitis in Cystic Fibrosis

2020

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Section: Clinical Considerations and Treatment Optionssupporting

confidence: 57%

Section: Inability To Suppress Neutrophil Activity In Pwcfmentioning

confidence: 99%

Vasculitis in Cystic Fibrosis

2020

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“…Pathologically, untreated KHE lesions are characterized by progressive fibrosis [12]. There is ample evidence for the crucial role of platelet activation and aggregation in the development of fibrosis in different tissues and organs [74,75]. It is conceivable that platelet activation and aggregation in muscles and connective tissues during KHE infiltration can activate variable fibrotic pathways.…”

Section: Musculoskeletal Disordersmentioning

confidence: 99%

Kaposiform hemangioendothelioma: current knowledge and future perspectives

Chen

Yang

et al. 2020

Orphanet J Rare Dis

115

180

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Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm with high morbidity and mortality. The initiating mechanism during the pathogenesis of KHE has yet to be discovered. The main pathological features of KHE are abnormal angiogenesis and lymphangiogenesis. KHEs are clinically heterogeneous and may develop into a lifethreatening thrombocytopenia and consumptive coagulopathy, known as the Kasabach-Merritt phenomenon (KMP). The heterogeneity and the highly frequent occurrence of disease-related comorbidities make the management of KHE challenging. Currently, there are no medications approved by the FDA for the treatment of KHE. Multiple treatment regimens have been used with varying success, and new clinical trials are in progress. In severe patients, multiple agents with variable adjuvant therapies are given in sequence or in combination. Recent studies have demonstrated a satisfactory efficacy of sirolimus, an inhibitor of mammalian target of rapamycin, in the treatment of KHE. Novel targeted treatments based on a better understanding of the pathogenesis of KHE are needed to maximize patient outcomes and quality of life. This review summarizes the epidemiology, etiology, pathophysiology, clinical features, diagnosis and treatments of KHE. Recent new concepts and future perspectives for KHE will also be discussed.

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“…PLT can release nitric oxide (NO) and prostanoids that control leukocyte interactions with endothelial cells (EC), respond to pathogens, and interact with blood leukocytes dictating their fate and functions (Rondina et al., 2013). Clinical-based and ex vivo evidence of increased PLT activation in CF has been documented, suggesting that these cells can contribute to inflammation in these patients (Ciabattoni et al., 2000; O’Sullivan et al., 2005; Lindberg et al., 2018) Moreover, PLTs are important effector of resolution, since they carry enzymes and substrates required for the biosynthesis of pro-resolving mediators. PLT interactions with PMN, a key process occurring during inflammation, lead to the formation of lipoxins (LX) A 4 (Romano and Serhan, 1992; Romano et al., 1993), the first identified specialized pro-resolving lipid mediator (SPM) that stops inflammation and promotes return to homeostasis (Romano et al., 2015).…”

Section: Non-resolving Airway Inflammation In Cystic Fibrosismentioning

confidence: 99%

Roles, Actions, and Therapeutic Potential of Specialized Pro-resolving Lipid Mediators for the Treatment of Inflammation in Cystic Fibrosis

2019

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Non-resolving inflammation is the main mechanism of morbidity and mortality among patients suffering from cystic fibrosis (CF), the most common life-threatening human genetic disease. Resolution of inflammation is an active process timely controlled by endogenous specialized pro-resolving lipid mediators (SPMs) produced locally in inflammatory loci to restrain this innate response, prevent further damages to the host, and permit return to homeostasis. Lipoxins, resolvins, protectins, and maresins are SPM derived from polyunsaturated fatty acids that limit excessive leukocyte infiltration and pro-inflammatory signals, stimulate innate microbial killing, and enhance resolution. Their unique chemical structures, receptors, and bioactions are being elucidated. Accruing data indicate that SPMs carry protective functions against unrelenting inflammation and infections in preclinical models and human CF systems. Here, we reviewed their roles and actions in controlling resolution of inflammation, evidence for their impairment in CF, and proofs of principle for their exploitation as innovative, non-immunosuppressive drugs to address inflammation and infections in CF.

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Increased platelet activation occurs in cystic fibrosis patients and correlates to clinical status

Cited by 24 publications

References 41 publications

Vasculitis in Cystic Fibrosis

Vasculitis in Cystic Fibrosis

Kaposiform hemangioendothelioma: current knowledge and future perspectives

Roles, Actions, and Therapeutic Potential of Specialized Pro-resolving Lipid Mediators for the Treatment of Inflammation in Cystic Fibrosis

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