title: Beyond hemostasis: the challenge of treating plasminogen deficiency. A report of three cases.
abstract: 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.
authors.affiliation: Unit of Hemostasis and Thrombosis, University Hospital Vall d'Hebron, Barcelona, Spain.|Department of Hematology, University Hospital Vall d'Hebron, Passeig Vall d' Hebron, 109-119, 08035, Barcelona, Spain.
authors.forename: Verónica
authors.initials: V
authors.lastname: Pons
authors.affiliation: Unit of Hemostasis and Thrombosis, University Hospital Vall d'Hebron, Barcelona, Spain.|Department of Hematology, University Hospital Vall d'Hebron, Passeig Vall d' Hebron, 109-119, 08035, Barcelona, Spain.
authors.forename: Pável
authors.initials: P
authors.lastname: Olivera
authors.affiliation: Unit of Hemostasis and Thrombosis, University Hospital Vall d'Hebron, Barcelona, Spain.|Department of Hematology, University Hospital Vall d'Hebron, Passeig Vall d' Hebron, 109-119, 08035, Barcelona, Spain.
authors.forename: Roberto
authors.initials: R
authors.lastname: García-Consuegra
authors.affiliation: Unit of Hemostasis and Thrombosis, University Hospital Vall d'Hebron, Barcelona, Spain.|Department of Hematology, University Hospital Vall d'Hebron, Passeig Vall d' Hebron, 109-119, 08035, Barcelona, Spain.
authors.forename: Laura
authors.initials: L
authors.lastname: López-Andreoni
authors.affiliation: Department of Ophthalmology, University Hospital Vall d'Hebron, Barcelona, Spain.
authors.forename: Nieves
authors.initials: N
authors.lastname: Martín-Begué
authors.affiliation: Department of Pathology, University Hospital Vall d'Hebron, Barcelona, Spain.
authors.forename: Angel
authors.initials: A
authors.lastname: García
authors.affiliation: Department of Hematology, University Hospital Vall d'Hebron, Passeig Vall d' Hebron, 109-119, 08035, Barcelona, Spain.
authors.forename: Juliana
authors.initials: J
authors.lastname: Hidalgo
authors.affiliation: Department of Hematology, University Hospital Vall d'Hebron, Passeig Vall d' Hebron, 109-119, 08035, Barcelona, Spain.
authors.forename: Francesc
authors.initials: F
authors.lastname: Bosch
authors.affiliation: Unit of Hemostasis and Thrombosis, University Hospital Vall d'Hebron, Barcelona, Spain. asantamaria@vhebron.net.|Department of Hematology, University Hospital Vall d'Hebron, Passeig Vall d' Hebron, 109-119, 08035, Barcelona, Spain. asantamaria@vhebron.net.
authors.forename: Amparo
authors.initials: A
authors.lastname: Santamaría
mesh_terms: D025861:Blood Coagulation Disorders, Inherited / Q000097:blood / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D002675:Child, Preschool; D003231:Conjunctivitis / Q000097:blood / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D005260:Female; D006399:Hematocolpos / Q000097:blood / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D010493:Pericarditis / Q000097:blood / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D010958:Plasminogen / Q000008:administration & dosage* / Q000172:deficiency*
chemical_list: D010958:Plasminogen
keywords: Ligneous conjunctivitis; Plasminogen deficiency