The first two Japanese cases of severe type I congenital plasminogen deficiency with ligneous conjunctivitis: successful treatment with direct thrombin inhibitor and fresh plasma.

Abstract:

:A 71-year-old woman and her elder sister developed ligneous conjunctivitis after ocular surgery. Laboratory tests demonstrated that the proband and her sister had 6.6% and 8.1% of plasminogen activity, and 1.2 and 1.4 mg/dl of antigen, respectively. Thus, they were diagnosed as having severe type I plasminogen deficiency, for the first time, in Japan. DNA sequencing and PCR-RFLP analyses revealed that these two cases are homozygotes of a novel A-to-G mutation at the obligatory splicing acceptor site in intron-C. Both cases were satisfactorily treated with a direct thrombin inhibitor, topical Argatroban, and topical plasma obtained from their healthy family members.

journal_name

Am J Hematol

authors

Suzuki T,Ikewaki J,Iwata H,Ohashi Y,Ichinose A

doi

10.1002/ajh.21402

subject

Has Abstract

pub_date

2009-06-01 00:00:00

pages

363-5

issue

6

eissn

0361-8609

issn

1096-8652

journal_volume

84

pub_type

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