Welcome to the Plasminogen Survey.

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Ligneous conjunctivitis was first described in 1847 by Bouisson, who noted pseudomembranes with a wood-like consistency covering the eye of a patient.[1] However, the term ligneous conjunctivitis, would not be coined until 1933.[1] In subsequent years, these pseudomembranes were found to involve the gingiva, ear, respiratory tract, female genitourinary tract, skin, and renal collecting system in affected individuals.[2] It was not until 1997 that plasminogen deficiency was firmly established as the etiology of these pseudomembranes. [3]

Unfortunately, much about plasminogen deficiency is unknown; chief among these is prevalence. One study of over 9000 blood donors in Scotland demonstrated a prevalence of 2.9/1000 heterozygous plasminogen deficient subjects, of which none were symptomatic.[4] This data generates a theoretical prevalence of homozygotes or compound-heterozygotes of 1.6/1,000,000.[1] Further epidemiological studies are required to determine the true prevalence of this condition. In addition, consensus diagnostic criteria are lacking and therapeutic options limited. Several factors contribute to difficulty in resolving these issues including waxing and waning of symptoms over time, variability of affected areas even within one family, and wide variety of medical specialties that represent the initial point of care for these patients.

Therefore, this survey serves as a starting point to collect information to begin to address these issues. The survey will collect information from a broad base of providers including the network of federally recognized comprehensive hemophilia treatment centers, and targeted specialties that may be caring or seeing patients with plasminogen deficiency including ophthalmology, oral surgery, otolaryngology, hematology, etc. It is hoped that this information will serve as a foundation for development of a network of health care providers that may be utilized for information sharing and for conduct of clinical research for new products targeted to plasminogen deficient patients.

The plasminogen deficiency survey is short and should require 10 to 15 minutes per subject. Please complete as much information as possible.

It will be helpful to have the patient's chart available during the survey process as some information regarding age and site of symptoms is requested, including information regarding fertility and pregnancy where applicable.

Please do not hesitate to contact us for any questions or concerns regarding this survey.

Rakesh Mehta, M.D. Amy Shapiro, M.D.
Assistant Professor of Clinical Medicine
Section of Hematology/Oncology
Indiana University School of Medicine
Medical Director
Indiana Hemophilia & Thrombosis Center
535 Barnhill Dr., RT- 473 8402 Harcourt Rd. Suite 420
Indianapolis, IN 46202 Indianapolis, IN 46260
Tel:317-278-9776 Tel: 317-871-0000, Toll Free: 877-CLOTTER (877-256-8837)
Fax: 317-274-3684 Fax: 317-871-0010
Email: Email: / Web:

[1]Schuster V, Seregard S. Ligneous conjunctivitis. Surv Ophthalmol. 2003;48:369-388.
[2]Schuster V, Hugle B, Tefs, K. Plasminogen Deficiency. J Thromb Haemost. 2077;5: 2315-2322.
[3]Mingers AM, Heimburger N, Zeitler P, Kreth HW, Schuster V. Homozygous type I plasminogen deficiency. Semin Thromb Hemost. 1997;23:259-269.
[4]Tait RC, Walker ID, Conkie JA, Islam SI, McCall F. Isolated familial plasminogen deficiency may not be a risk factor for thrombosis. Thromb Haemost. 1996;76:1004-1008.
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