Congenital plasminogen deficiency with long standing pseudomembranous conjunctival and genital lesions

Magdaleno‐Tapial, Jorge; Hernández-Bel, P.; Valenzuela‐Oñate, Cristian; Gimeno-Ferrer, Fátima; Rodríguez‐López, Raquel; Hernández-Bel, Laura; Sabater‐Marco, Vicente; Miquel, V. Alegre-de

doi:10.1016/j.jdcr.2018.09.004

Cited by 4 publications

(3 citation statements)

References 8 publications

(8 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Evidently, plasmin-mediated breakdown of blood clots is required for a healthy vasculature; hypofibrinolysis is a risk factor for venous thromboembolism (72). Although prophylactic treatment of HAE patients with antifibrinolytic agents is well-tolerated, human plasminogen deficiency is associated with development of ligneous conjunctivitis (73). This suggests that generalized neutralization of plasminogen activation might have adverse effects.…”

Section: Therapeutic Targeting Of Plasminflammationmentioning

confidence: 99%

Plasminflammation—An Emerging Pathway to Bradykinin Production

Maas

2019

Front. Immunol.

View full text Add to dashboard Cite

Plasminogen activation is essential for fibrinolysis—the breakdown of fibrin polymers in blood clots. Besides this important function, plasminogen activation participates in a wide variety of inflammatory conditions. One of these conditions is hereditary angioedema (HAE), a rare disease with characteristic attacks of aggressive tissue swelling due to unregulated production and activity of the inflammatory mediator bradykinin. Plasmin was already implicated in this disease decades ago, but a series of recent discoveries have made it clear that plasmin actively contributes to this pathology. Collective evidence points toward an axis in which the plasminogen activation system and the contact system (which produces bradykinin) are mechanistically coupled. This is amongst others supported by findings in subtypes of HAE that are caused by gain-of-function mutations in the genes that respectively encode factor XII or plasminogen, as well as clinical experience with the antifibrinolytic agents in HAE. The concept of a link between plasminogen activation and the contact system helps us to explain the inflammatory side effects of fibrinolytic therapy, presenting as angioedema or tissue edema. Furthermore, these observations motivate the development and characterization of therapeutic agents that disconnect plasminogen activation from bradykinin production.

show abstract

Section: Therapeutic Targeting Of Plasminflammationmentioning

confidence: 99%

Plasminflammation—An Emerging Pathway to Bradykinin Production

Maas

2019

Front. Immunol.

View full text Add to dashboard Cite

show abstract

“…Awareness about plasminogen deficiency and its symptoms are essential for early diagnosis and treatment of this challenging disease [ 9 ]. Currently, plasma-derived plasminogen concentrate is reported to resolve all complications, except infertility [6].…”

Section: To the Editormentioning

confidence: 99%

A Peculiar Disease of a Young Woman who Wants to Get Pregnant

Çelkan

Fenercioğlu

Kaçar

2021

Tjh

View full text Add to dashboard Cite

“…Notably, while plasminogen deficiency is usually diagnosed in infancy or childhood, gynecologic disease differs in that most patients present in adulthood, usually with a history of infertility, dysmenorrhea or abnormal vaginal bleeding (4). Postmenopausal presentation has also been recorded (5,6).…”

mentioning

confidence: 99%

Atypical Microglandular Hyperplasia of Endocervix as the Presenting Feature of Plasminogen Deficiency

Stewart¹

2020

International Journal of Gynecological Pathology

View full text Add to dashboard Cite

A 30-yr-old patient with no significant past medical history presented with postcoital bleeding and was found to have fibrinous pseudomembranous lesions overlying and partly in continuity with the endocervical mucosa. Histologically, these were characterized by an atypical microglandular proliferation that was associated with extensive fibrinous exudate and a prominent neutrophil polymorph infiltrate. Ligneous stromal alteration was not identified but the changes prompted hematologic review which confirmed plasminogen deficiency. A subsequent endometrial biopsy also demonstrated degenerate glands within a fibrin-rich matrix. This is the third case demonstrating an association between atypical endocervical microglandular hyperplasia and plasminogen deficiency. The diagnosis should also be considered when biopsies demonstrate exuberant fibrin exudate even if ligneous disease is not present.

show abstract

Congenital plasminogen deficiency with long standing pseudomembranous conjunctival and genital lesions

Cited by 4 publications

References 8 publications

Plasminflammation—An Emerging Pathway to Bradykinin Production

Plasminflammation—An Emerging Pathway to Bradykinin Production

A Peculiar Disease of a Young Woman who Wants to Get Pregnant

Atypical Microglandular Hyperplasia of Endocervix as the Presenting Feature of Plasminogen Deficiency

Contact Info

Product

Resources

About