Classification of Abnormal Plasminogens: Dysplasminogenemias

Robbins, Kenneth C.

doi:10.1055/s-2007-1002672

Cited by 15 publications

(19 citation statements)

References 12 publications

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“…In this study, c. 27% of individuals with dysplasminogenaemia reported a clinical history of thrombosis. A number of additional PLG polymorphisms and clinically significant dysplasminogenaemias have also been reported (Robbins, 1990).…”

Section: Developmental Regulation and Disorders Of Plasmin Generationmentioning

confidence: 99%

Molecular mechanisms of fibrinolysis

2005

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SummaryThe molecular mechanisms that finely co-ordinate fibrin formation and fibrinolysis are now well defined. The structure and function of all major fibrinolytic proteins, which include serine proteases, their inhibitors, activators and receptors, have been characterized. Measurements of real time, dynamic molecular interactions during fibrinolysis of whole blood clots can now be carried out in vitro. The development of genetargeted mice deficient in one or more fibrinolytic protein(s) has demonstrated expected and unexpected roles for these proteins in both intravascular and extravascular settings. In addition, genetic analysis of human deficiency syndromes has revealed specific mutations that result in human disorders that are reflective of either fibrinolytic deficiency or excess. Elucidation of the fine control of fibrinolysis under different physiological and pathological haemostatic states will undoubtedly lead to novel therapeutic interventions. Here, we review the fundamental features of intravascular plasmin generation, and consider the major clinical syndromes resulting from abnormalities in fibrinolysis.

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Section: Developmental Regulation and Disorders Of Plasmin Generationmentioning

confidence: 99%

Molecular mechanisms of fibrinolysis

2005

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“…In dysplasminogenemia, or type II plg deficiency, the level of immunoreactive plg is normal (or only slightly reduced), whereas the specific functional plg activity is markedly reduced because of abnormalities in the variant plg molecule [73].…”

Section: Dysplasminogenemia (Type II Plg Deficiency)mentioning

confidence: 99%

“…The described abnormal plg was later referred to as plg Tochigi I (Table 1), the most common cause of dysplasminogenemia in Japan and other Asian countries. Following this, several other less common abnormal plg variants leading to dysplasminogenemia have been identified and referred to as plg variants: Nagoya, Osaka, Kanagawa, Kagoshima, Tokyo, Chicago, Frankfurt and Maywood [73]. In some of these, the molecular genetic defect has been identified (Table 1, Fig.…”

Section: Dysplasminogenemia (Type II Plg Deficiency)mentioning

confidence: 99%

Plasminogen deficiency

Schuster

Hügle

Tefs

2007

Journal of Thrombosis and Haemostasis

164

160

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Summary. Plasminogen (plg) deficiency has been classified as (i) hypoplasminogenemia or ‘true’ type I plg deficiency, and (ii) dysplasminogenemia, also called type II plg deficiency. Both forms, severe hypoplasminogenemia and dysplasminogenemia, are not causally linked to venous thrombosis. Dysplasminogenemia does not lead to a specific clinical manifestation and probably represents only a polymorphic variation in the general population, mainly in Asian countries. Severe hypoplasminogenemia is associated with compromised extracellular fibrin clearance during wound healing, leading to pseudomembraneous (ligneous) lesions on affected mucous membranes (eye, middle ear, mouth, pharynx, duodenum, upper and lower respiratory tract and female genital tract). Ligneous conjunctivitis is by far the most common clinical manifestation. More than 12% of patients with severe hypoplasminogenemia exhibit congenital occlusive hydrocephalus. In milder cases of ligneous conjunctivitis, topical application of plg‐containing eye drops, fresh frozen plasma, heparin, corticosteroids or certain immunosuppressive agents (such as azathioprine) may be more or less effective. Oral treatment with sex hormones was successful in two female patients with ligneous conjunctivitis. In severe cases with possibly life‐threatening multi‐organ involvement, true therapeutic options are not available at present. The plg‐knockout mouse is a useful tool to study the many different properties of plg in a variety of settings, such as wound healing, tissue repair and tissue remodeling, virulence and invasiveness of certain bacteria in the human host, tumor growth and dissemination, as well as arteriosclerosis.

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“…In dysplasminogenemia, despite a normal immunoreactive plasminogen level, functional activity is markedly decreased due to abnormalities in the variant PLG molecule (7). Dysplasminogenemia seems to be more prevalent in Asia.…”

Section: Discussionmentioning

confidence: 99%

A Case of Acute Pulmonary Embolism Associated with Dysplasminogenemia

Yoo

Kim

et al. 2013

J Korean Med Sci

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The incidence of pulmonary embolism (PE) rises markedly with age, and only a few cases have been reported in younger adults. Thrombophilia has been reported as one of the predisposing factors for PE in younger adults. Here we report an extraordinary case of PE complicated with dysplasminogenemia, a rare genetic disorder resulting in hypercoagulability, in a young male. An 18-yr-old male visited an emergency room in the United States complaining chest discomfort. He was diagnosed as PE with deep vein thrombosis without apparent risk factors. Anticoagulation therapy with warfarin had been initiated and discontinued after 6 months of treatment. After returning to Korea he was tested for thrombophilia which revealed decreased activity of plasminogen and subsequent analysis of PLG gene showed heterozygous Ala620Thr mutation. He was diagnosed with PE complicated with dysplasminogenemia. Life-long anticoagulation therapy was initiated. He is currently under follow-up without clinical events for 2 yr.

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Classification of Abnormal Plasminogens: Dysplasminogenemias

Cited by 15 publications

References 12 publications

Molecular mechanisms of fibrinolysis

Molecular mechanisms of fibrinolysis

Plasminogen deficiency

A Case of Acute Pulmonary Embolism Associated with Dysplasminogenemia

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