Hereditary prothrombin deficiency is an autosomal recessive disorder with an estimated incidence of 1 in 2 million . Presentation of the disease is variable; however, it is usually associated with moderate to severe bleeding tendencies including muscle hematomas, hemarthrosis, intracranial, mucosal, and postoperative bleeding. Here we report a case of a 35-year-old pregnant woman with congenital hypoprothrombinemia and idiopathic thrombocytopenic purpura, review the literature, and discuss its epidemiology, presentation, diagnosis, and treatment.
Introduction Hereditary thrombophilia describes an inherited tendency to form venous or arterial thrombosis second to loss of natural anticoagulants. The optimal patient populations to test for these thrombophilic states are controversial. No accepted guidelines exist for whom to test, but consensus among many experts is that a targeted approach should be taken. Both the College of American Pathologists (CAP) and the American College of Medical Genetics (ACMG) suggest testing the following patients: Idiopathic VTE at <50 years old, recurrent VTE, and unusual sites of VTE (e.g., mesenteric, portal, hepatic) [1,2]. Hypercoagulable testing should not be done during acute thrombosis or while receiving anticoagulation. Given our clinical observations, we hypothesized that hypercoagulable testing is often done without the proper indication and performed at an inappropriate time at our institution. A single institution study was performed at our hospital from January '13 through August '13 reviewing inpatients that had Factor V Leiden and Prothrombin Gene mutations, Antithrombin, Protein C, and Protein S antigens or activities ordered, and evaluated as to whether or not they were ordered in concordance with the CAP and ACMG indications. Results from that study illustrated that of the 43 patients that had these tests ordered; only 13 were ordered based on appropriate indications from CAP and ACMG. Of those 13 patients, all were tested at an inappropriate time, either during an acute thrombosis or while on anticoagulation. The departments responsible for ordering the majority of the tests were Medicine, Neurology and Ob/Gyn., making up greater than 75% of the orders. In view of this information we designed an intervention to further educate the staff and to evaluate its outcome. Methods From September '14 through December '14, we educated the staff responsible for ordering these tests via instructional seminars. Each department was educated separately, and was given 30 minute lectures reviewing the utilities, indications and appropriate timing of ordering a thrombophilic workup. This was repeated several times over the course of the three month period. We then retrospectively reviewed charts of hospitalized patients at our hospital from January '15 through August '15. A patient must have had the following tests ordered to be included in the study: Factor V Leiden mutation, Prothrombin Gene mutation, Antithrombin, Protein C, and Protein S antigens or activities. Data compiled from electronic databases included age, clinical indication for hypercoagulable workup, ordering service, and whether or not anticoagulation was present during testing. These indications were then compared to the indications recommended by both the CAP and the ACMG listed above. If testing occurred for a non-approved indication, during acute thrombosis, or while patients were receiving anticoagulation it was deemed inappropriate. Results 22 patients had inpatient hypercoaguable testing sent over the time period of January '15 through June '15 as compared to 43 tested from January '13 through August '13, prior to the intervention. Of the 22 patients, 3/22 (14%) were tested appropriately according to the CAP and ACMG recommendations. However, all 3 patients had hypercoaguable testing sent inappropriately when timing was analyzed; all were tested during an acute thrombosis or while on anticoagulation. When comparing the current data to that prior to the intervention, there was a trend of decreasing number of hypercoaguable tests ordered. The departments given the seminar all had a decreasing trend in tests ordered. Despite this, the majority of the tests ordered after the interventions were still ordered incorrectly or while either on anticoagulant treatment or during an acute thrombosis. Discussion Hypercoagulable testing is being over utilized in the inpatient setting, largely because it is being performed for inappropriate indications and during suboptimal conditions. Principally, this is due to lack of knowledge on the indications and timing of ordering these thrombophilic tests. Here we demonstrate how education, in the form of lectures and seminars, can inform the staff on how and when to order these hypercoaguable panels. This study acts as a template to illustrate how education in the form of brief and repeated seminars can help change practice habits, provide better quality care and prevent inappropriate testing. Disclosures No relevant conflicts of interest to declare.
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