Type I plasminogen deficiency with unexpected clinical aspects: Could be more than coexistence?

Altıner, Şule; Klammt, J; Bernhard, Matthias K.; Schuster, Volker; Karabulut, Halil Gürhan

doi:10.1080/2331205x.2017.1334317

Cited by 2 publications

(3 citation statements)

References 14 publications

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“…1,2 Patients with severe hypoplasminogenemia display clinical symptoms of a persistent inflammatory state with defective wound healing, lower body weight, and reduced fertility. [3][4][5][6][7] The etiology behind these clinical symptoms is linked to the formation of persistent fibrin deposits in mucous pseudomembranes. 3,4,7 The prognosis is highly variable, and managing the disorder is challenging due to varying clinical symptoms, the multisystem manifestations, and the lack of effective treatments.…”

Section: Introductionmentioning

confidence: 99%

“…[3][4][5][6][7] The etiology behind these clinical symptoms is linked to the formation of persistent fibrin deposits in mucous pseudomembranes. 3,4,7 The prognosis is highly variable, and managing the disorder is challenging due to varying clinical symptoms, the multisystem manifestations, and the lack of effective treatments. [6][7][8][9][10] Genetically modified mice with complete plasminogen deficiency have been used to study the disorder.…”

Section: Introductionmentioning

confidence: 99%

“…3,4,7 The prognosis is highly variable, and managing the disorder is challenging due to varying clinical symptoms, the multisystem manifestations, and the lack of effective treatments. [6][7][8][9][10] Genetically modified mice with complete plasminogen deficiency have been used to study the disorder. [3][4][5][11][12][13][14] Similar to the patients, plasminogen-deficient mice (Plg 2/2 ) exhibit spontaneous and persistent extravascular fibrin deposits in multiple organ systems.…”

Section: Introductionmentioning

confidence: 99%

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Absence of functional compensation between coagulation factor VIII and plasminogen in double-knockout mice

et al. 2018

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Plasminogen deficiency is associated with severely compromised fibrinolysis and extravascular deposition of fibrin. In contrast, coagulation factor VIII (FVIII) deficiency leads to prolonged and excessive bleeding. Based on opposing biological functions of plasminogen and FVIII deficiencies, we hypothesized that genetic elimination of FVIII would alleviate the systemic formation of fibrin deposits associated with plasminogen deficiency and, in turn, elimination of plasminogen would limit bleeding symptoms associated with FVIII deficiency. Mice with single and combined deficiencies of FVIII (F8−/−) and plasminogen (Plg−/−) were evaluated for phenotypic characteristics of plasminogen deficiency, including wasting disease, shortened lifespan, rectal prolapse, and multiorgan fibrin deposition. Conversely, to specifically examine the role of plasmin-mediated fibrinolysis on bleeding caused by FVIII deficiency, F8−/− and F8−/−/Plg−/− mice were subjected to a bleeding challenge. Mice with a combined deficiency in FVIII and plasminogen displayed no phenotypic differences relative to mice with single FVIII or plasminogen deficiency. Plg−/− and F8−/−/Plg−/− mice exhibited the same penetrance and severity of wasting disease, rectal prolapse, extravascular fibrin deposits, and reduced viability. Furthermore, following a tail vein–bleeding challenge, no significant differences in bleeding times or total blood loss could be detected between F8−/− and F8−/−/Plg−/− mice. Moreover, F8−/− and F8−/−/Plg−/− mice responded similarly to recombinant FVIII (rFVIII) therapy. In summary, the pathological phenotype of Plg−/− mice developed independently of FVIII-dependent coagulation, and elimination of plasmin-driven fibrinolysis did not play a significant role in a nonmucosal bleeding model in hemophilia A mice.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Absence of functional compensation between coagulation factor VIII and plasminogen in double-knockout mice

et al. 2018

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Plasminogen Deficiency: A Case Report and Review

Nasiri,

Nassar,

Alzahrani

2023

Cureus

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Plasminogen deficiency, a rare disorder characterized by impaired fibrinolysis, frequently results in ligneous conjunctivitis. In this report, we report a case of a Saudi girl manifesting both conjunctivitis and hydrocephalus. Her initial symptoms at 1 month of age were recurring eye redness, which was inaccurately diagnosed as simple conjunctivitis. Surgical intervention for her ocular lesions revealed underlying membrane deposition. She later exhibited signs of increased intracranial pressure, resulting in a hydrocephalus diagnosis and subsequent surgery. Genetic analysis confirmed the presence of plasminogen deficiency. Clinical evaluations highlighted ligneous conjunctivitis, variations in visual acuity, and facial acne. Laboratory assessments demonstrated diminished plasminogen levels. The therapeutic approach encompassed plasminogen replacement, administered intravenously (1000 units, thrice weekly) and as eye drops, with the potential addition of fresh frozen plasma. Notably, this replacement therapy led to a significant reduction in hospital admissions and the severity of her conjunctivitis. Given the challenges in procuring consistent plasminogen supplies, the viability of hepatic transplantation is currently under investigation.

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Type I plasminogen deficiency with unexpected clinical aspects: Could be more than coexistence?

Cited by 2 publications

References 14 publications

Absence of functional compensation between coagulation factor VIII and plasminogen in double-knockout mice

Absence of functional compensation between coagulation factor VIII and plasminogen in double-knockout mice

Plasminogen Deficiency: A Case Report and Review

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