Beyond hemostasis: the challenge of treating plasminogen deficiency. A report of three cases

Congenital plasminogen deficiency is a rare autosomal recessive disorder, characterized by chronic mucosal membranous lesions. Although the most common clinical manifestation is eye involvement as ligneous conjunctivitis, extra-ocular lesions affecting other mucosal surfaces indicates a systemic dis...

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Published inJournal of thrombosis and thrombolysis Vol. 41; no. 3; pp. 544 - 547
Main Authors Pons, Verónica, Olivera, Pável, García-Consuegra, Roberto, López-Andreoni, Laura, Martín-Begué, Nieves, García, Angel, Hidalgo, Juliana, Bosch, Francesc, Santamaría, Amparo
Format Journal Article
LanguageEnglish
Published New York Springer US 01.04.2016
Springer Nature B.V
Subjects
Blood Coagulation Disorders, Inherited - blood
Blood Coagulation Disorders, Inherited - drug therapy
Blood Coagulation Disorders, Inherited - genetics
Blood Coagulation Disorders, Inherited - pathology
Cardiology
Child, Preschool
Conjunctivitis - blood
Conjunctivitis - drug therapy
Conjunctivitis - genetics
Conjunctivitis - pathology
Female
Hematocolpos - blood
Hematocolpos - drug therapy
Hematocolpos - genetics
Hematocolpos - pathology
Hematology
Humans
Male
Medicine
Medicine & Public Health
Middle Aged
Pericarditis - blood
Pericarditis - drug therapy
Pericarditis - genetics
Pericarditis - pathology
Plasminogen - administration & dosage
Plasminogen - deficiency
Ligneous conjunctivitis
Plasminogen deficiency
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Summary:Congenital plasminogen deficiency is a rare autosomal recessive disorder, characterized by chronic mucosal membranous lesions. Although the most common clinical manifestation is eye involvement as ligneous conjunctivitis, extra-ocular lesions affecting other mucosal surfaces indicates a systemic disease. In this report we describe two cases with atypical extra-ocular involvement that includes pericarditis and recurrent hematocolpos, and one with paradoxical correlation between ocular lesions and plasminogen levels. In ligneous conjunctivitis, although different treatment strategies have been tried with mild success, the only effective therapy is topical or systemic plasminogen concentrates that are not commercially available. Unfortunately there is not either effective management for cases with multisystemic disease. Hence, treatment for plasminogen deficiency is still a challenge and the variability of the clinical spectrum in this pathology makes necessary a multidisciplinary approach.
Bibliography:ObjectType-Case Study-2
SourceType-Scholarly Journals-1
ObjectType-Feature-4
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ObjectType-Report-1
ObjectType-Article-3
ISSN:0929-5305
1573-742X
DOI:10.1007/s11239-015-1225-3