Congenital Abnormal Plasminogen, Frankfurt I, a Cause for Recurrent Venous Thrombosis

Scharrer, I.; Hach-Wunderle, V.; Wohl, Robert C.; Sinio, Leonida; Boreisha, Irena G.; Robbins, Kenneth C.

doi:10.1159/000215841

Cited by 5 publications

(3 citation statements)

References 10 publications

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“…Originally, several older studies have linked dysplasminogenemia with an increased risk for deep vein thrombosis [74,80,81]. However, later and more comprehensive studies have clearly demonstrated that isolated dysplasminogenemia is not a risk factor for thrombosis [26,82].…”

Section: Dysplasminogenemia (Type II Plg Deficiency)mentioning

confidence: 99%

Plasminogen deficiency

Schuster

Hügle

Tefs

2007

Journal of Thrombosis and Haemostasis

165

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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Section: Dysplasminogenemia (Type II Plg Deficiency)mentioning

confidence: 99%

Plasminogen deficiency

Schuster

Hügle

Tefs

2007

Journal of Thrombosis and Haemostasis

165

160

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“…Similarly abnormally elevated levels of histidine-rich glycoprotein or increased levels of alpha-2-antiplasmin are extremely uncommon in familial thrombophil ia (9,10). In contrast, the first reports linking familial dysplasminogenaemia (11) and hypoplasminogenaemia (12) with thrombosis were quickly corroborated by many similar publications (13)(14)(15)(16)(17)(18)(19)(20). However, in a review of 20 such families, Dolan and Preston (21) found that only one of 61 affected relatives had suffered a thrombosis.…”

Section: Introductionmentioning

confidence: 99%

Isolated Familial Plasminogen Deficiency May not Be a Risk Factor for Thrombosis

et al. 1996

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SummaryDespite many reports of individuals with congenital plasminogen deficiency and thrombosis, there is still uncertainty whether heterozygous deficiency represents a real thrombophilic risk factor or simply a coincidental finding. We have addressed this issue by testing for plasminogen deficiency in a cohort of 9611 blood donors. Out of 66 donors with reduced plasminogen activity on two occasions 28 were shown to have a familial deficiency state (including 3 with dysplasminogen-aemia). Our observed prevalence rate for familial plasminogen deficiency, calculated at 2.9/1000 (95% Cl = 1.9-4.2 per 1000), was not significantly different from that calculated from published reports of congenital plasminogen deficiency in thrombotic cohorts (5.4/1000). Furthermore, with only two exceptions, all 80 donors and relatives with familial deficiency were asymptomatic with regard to thrombosis -including a 29 year old donor with suspected compound heterozygous hypoplasminogenaemia. These findings add further weight to the argument that familial heterozygous plasminogen deficiency, at least in isolation, does not constitute a significant thrombotic risk factor. However, it remains uncertain whether plasminogen deficiency, when combined with other thrombophilic conditions, may become more clinically important.

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“…In recent years there has been much interest in the association between venous thrombosis and reduced levels of functioning PLG resulting from either a dysplasminogenaemic (1)(2)(3)(4)(5) or hypoplasminogenaemic state (6)(7)(8)(9)(10). Numerous cases of familial PLG deficiency have been described (10)(11)(12)(13)(14)(15)(16)(17), however, the link with thrombosis is tenuous since the propositus is often the only symptomatic family member.…”

Section: Introductionmentioning

confidence: 99%

Plasminogen Levels in Healthy Volunteers -Influence of Age, Sex, Smoking and Oral Contraceptives

et al. 1992

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SummaryThere is considerable doubt as to the importance of reduced plasminogen (PLG) activity as a risk factor for venous thrombosis. In the present study we have identified a wide range of PLG activities (25–200%) in a cohort of 9,611 blood donors. Males and females not taking hormonal contraceptives show a similar distribution of PLG, however, variation related to age appears to follow a different pattern in males and females. These differences are of doubtful clinical importance as are differences related to smoking. In contrast, females taking hormonal contraceptives or hormone replacement therapy (HRT) have up to 25% higher mean PLG levels in younger females but a less marked elevation (10%) is seen in 40–50 year olds. A PLG activity <65% was recorded in 61 donors, none of whom appeared to have a history of thrombosis. These findings do not support the notion that reduced PLG is an important thrombophilic risk factor, however, further investigation of the donors with low PLG is required.

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Congenital Abnormal Plasminogen, Frankfurt I, a Cause for Recurrent Venous Thrombosis

Cited by 5 publications

References 10 publications

Plasminogen deficiency

Plasminogen deficiency

Isolated Familial Plasminogen Deficiency May not Be a Risk Factor for Thrombosis

Plasminogen Levels in Healthy Volunteers -Influence of Age, Sex, Smoking and Oral Contraceptives

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