Beyond hemostasis: the challenge of treating plasminogen deficiency. A report of three cases
Por:
Pons, V, Olivera, P, Garcia-Consuegra, R, Lopez-Andreoni, L, Martin-Begu, N, Garcia, A, Hidalgo, J, Bosch, F and Santamaria, A
Publicada:
1 abr 2016
Resumen:
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.
Filiaciones:
Pons, V:
Univ Hosp Vall dHebron, Unit Hemostasis & Thrombosis, Barcelona 08035, Spain
Univ Hosp Vall dHebron, Dept Hematol, Passeig Vall Hebron 109-119, Barcelona 08035, Spain
Olivera, P:
Univ Hosp Vall dHebron, Unit Hemostasis & Thrombosis, Barcelona 08035, Spain
Univ Hosp Vall dHebron, Dept Hematol, Passeig Vall Hebron 109-119, Barcelona 08035, Spain
Garcia-Consuegra, R:
Univ Hosp Vall dHebron, Unit Hemostasis & Thrombosis, Barcelona 08035, Spain
Univ Hosp Vall dHebron, Dept Hematol, Passeig Vall Hebron 109-119, Barcelona 08035, Spain
Lopez-Andreoni, L:
Univ Hosp Vall dHebron, Unit Hemostasis & Thrombosis, Barcelona 08035, Spain
Univ Hosp Vall dHebron, Dept Hematol, Passeig Vall Hebron 109-119, Barcelona 08035, Spain
Martin-Begu, N:
Univ Hosp Vall dHebron, Dept Ophthalmol, Barcelona 08035, Spain
Garcia, A:
Univ Hosp Vall dHebron, Dept Pathol, Barcelona 08035, Spain
Hidalgo, J:
Univ Hosp Vall dHebron, Dept Hematol, Passeig Vall Hebron 109-119, Barcelona 08035, Spain
Bosch, F:
Univ Hosp Vall dHebron, Dept Hematol, Passeig Vall Hebron 109-119, Barcelona 08035, Spain
:
Univ Hosp Vall dHebron, Unit Hemostasis & Thrombosis, Barcelona 08035, Spain
Univ Hosp Vall dHebron, Dept Hematol, Passeig Vall Hebron 109-119, Barcelona 08035, Spain
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