Classical complement pathway activation in immune thrombocytopenia purpura: inhibition by a novel C1s inhibitor

Peerschke, Ellinor I.B.; Panicker, Sandip; Bussel, James B.

doi:10.1111/bjh.13648

Cited by 37 publications

(31 citation statements)

References 9 publications

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“…The classical complement pathway is an important driver in the pathogenesis of HAE. Increasing evidence suggests a contribution of complement activation in ITP [ 17 , 18 ], the additional diagnosis for our patient. It could be considered that the disturbance of the coagulation system might increase the consumption of C1 and C1-INH and hereby act as a contributing cause in the development of HAE attacks.…”

Section: Discussionmentioning

confidence: 74%

Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

Andersen

Bygum

2015

Case Reports in Dermatological Medicine

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Hereditary angioedema is a rare, but potentially life-threatening genetic disorder that results from an autosomal dominant trait. It is characterized by acute, recurrent attacks of severe local edema, most commonly affecting the skin and mucosa. Swelling in hereditary angioedema patients does however not always have to be caused by angioedema but can relate to other concomitant disorders. In this report we are focusing on misdiagnosis in a patient with known hereditary angioedema, whose bleeding episode caused by idiopathic thrombocytopenic purpura was mistaken for an acute attack of hereditary angioedema. The case illustrates how clinicians can have difficulties in handling patients with rare diseases, especially in the emergency care setting.

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Section: Discussionmentioning

confidence: 74%

Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

Andersen

Bygum

2015

Case Reports in Dermatological Medicine

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“…Thus, the development of pathway-specific complement inhibitors has been a long-lasting goal over the past few decades. Compstatin, Compstatin 40 (Cp40), and its long-acting analog, polyethylene glycol(PEG-Cp40) are newly designed complement inhibitors that display inhibition on the upstream of the complement cascade ( 92 ). Compstatin is a cyclic fibrin polypeptide composed of 13 amino acids that binds to C3 and C3b.…”

Section: Therapeutic Relevance Of the Complement Systemmentioning

confidence: 99%

Complement in Hemolysis- and Thrombosis- Related Diseases

Luo

Wang

et al. 2020

Front. Immunol.

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The complement system, originally classified as part of innate immunity, is a tightly self-regulated system consisting of liquid phase, cell surface, and intracellular proteins. In the blood circulation, the complement system, platelets, coagulation system, and fibrinolysis system form a close and complex network. They activate and regulate each other and jointly mediate immune monitoring and tissue homeostasis. The dysregulation of each cascade system results in clinical manifestations and the progression of different diseases, such as sepsis, atypical hemolytic uremic syndrome, C3 glomerulonephritis, systemic lupus erythematosus, or ischemia-reperfusion injury. In this review, we summarize the crosstalk between the complement system, platelets, and coagulation, provide integrative insights into how complement dysfunction leads to hemopathic progression, and further discuss the therapeutic relevance of complement in hemolytic and thrombotic diseases.

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“…Three pathways have been identified for complement activation, which are the classical, alternative, and lectin pathway (Merle, Church, Fremeaux‐Bacchi, & Roumenina, ; Takahashi et al, ). IgG or IgM antigen/antibody complexes are responsible for initiating the classical pathway through binding to the first protein of the cascade (C1q) which in turn activates the C1r, leading to formation of the membrane attack complex which eventually penetrates bacterial membranes creating pores which lead to bacterial lysis (Peerschke, Panicker, & Bussel, ).…”

Section: Immune Systemmentioning

confidence: 99%

Modulation of innate and adaptive immune responses by arabinoxylans

Fadel

Plunkett

et al. 2017

J Food Biochem

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Humans are exposed to harmful pathogens and a wide range of noxious substances every day.The immune system reacts to, and destroys, these pathogens and harmful substances. The immune system is composed of innate and adaptive immunity, which liaise to protect the host and maintain health. Foods, especially cereals, have been reported to modulate the immune response.Arabinoxylans are nonstarch polysaccharides that have been shown to possess immunemodulatory activities. This review article discusses the fundamentals of the immune system and provides an overview of the immunomodulatory potential of arabinoxylans in conjunction with their structural characteristics and proposed similarities with lipopolysaccharides. Practical applicationsUnderstanding how the immune system works is of vital importance to prevent unnecessary or excessive inflammatory responses. Consumption of arabinoxylans has been shown to possess immunomodulatory potential. However, their mechanism of action has not been elucidated.Arabinoxylans share some similarities with lipopolysaccharides (LPS), a molecule that induces substantial and sometimes excessive immune responses such as fever following infection by pathogens. Thus, we propose that arabinoxylans might possibly act on the same receptor as LPS.Competition between dietary-derived arabinoxylans and LPS at a shared receptor would then have the potential to inhibit or attenuate excessive LPS-induced inflammatory responses that are typical of infection/fever. In the absence of infection and consequently no competition at the LPS receptor, consumption of dietary arabinoxylans may protect against the risk of infection by moderately activating the receptor and heightening natural (background) levels of immunity.

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Classical complement pathway activation in immune thrombocytopenia purpura: inhibition by a novel C1s inhibitor

Cited by 37 publications

References 9 publications

Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

Complement in Hemolysis- and Thrombosis- Related Diseases

Modulation of innate and adaptive immune responses by arabinoxylans

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