Treatment of Single Factor Deficiencies: A Case Study Approach

Marisa B Marques

doi:10.29074/ascls.16.2.120

This article requires a subscription to view the full text. If you have a subscription you may use the login form below to view the article. Access to this article can also be purchased.

Marisa B Marques, MD⇑
1. is the coagulation service medical director at the University of Alabama at Birmingham, Birmingham AL

Address for correspondence: Marisa B Marques MD, Department of Pathology, University of Alabama at Birmingham, 619 South 19th Street, West Pavilion, P230, Birmingham AL 35233. http://uabcoag.net

Extract

von Willebrand disease and hemophilia A are the two most common inherited bleeding disorders.¹ The diagnosis, treatment, and monitoring of patients with these conditions requires frequent interaction between the patient's physician and the clinical laboratory. In many institutions, coagulation factor concentrates are dispensed from the blood bank along with other blood-derived products, while the special coagulation laboratory offers assays to monitor response to treatment. It is important in these instances that there is open communication among the staff of those laboratories and an understanding of the important issues in the management of patients with von Willebrand disease or hemophilia A with or without inhibitor.² In this discussion, such issues are highlighted using a case study-based approach.

CASE 1: von WILLEBRAND DISEASE A 40-year old white female was hospitalized to have left total knee arthroplasty on the next day. Her past medical history was significant for severe von Willebrand disease and multiple episodes of hemarthrosis in the affected joint. The orthopedic surgeon called the coagulation consultant to decide on von Willebrand factor replacement for surgery.

Although von Willebrand disease is classically associated with manifestations of abnormal primary hemostasis such as mucosal bleeding, in the severe form of this disease, patients also have a deficiency of factor VIII and suffer from joint bleeds. The deficiency of factor VIII is a consequence of increased proteolysis of this factor in the absence of normal levels of von Willebrand factor to protect it in the circulation. Until a few years ago, cryoprecipitate was the product…