With advances in care, increasing numbers of people with hemophilia (PWH) achieve near-normal life expectancies and present with typical age-related cardiovascular conditions. Evidence-based guidelines for medical or surgical management of cardiovascular conditions in individuals with hemophilia are limited. Published recommendations exist for the management of some common cardiovascular conditions (eg, ischemic heart disease, atrial fibrillation), but identifying optimal strategies for anticoagulant or antithrombotic therapy constitutes the primary challenge of managing nonoperative cardiovascular disease (CVD) in PWH. In general, as long as factor concentrates or other hemostatic therapies maintain adequate hemostasis, the recommended medical and surgical management of CVD in PWH parallels that in individuals without hemophilia. The presence of factor inhibitors complicates hemophilia management. Published outcomes of CVD treatment in PWH are similar to those in the general population. Specific knowledge about factor replacement, factor inhibitors, and disease-specific treatment distinguishes the cardiovascular care of PWH from similar care of individuals without this rare bleeding disorder. Furthermore, a multidisciplinary approach incorporating a hematologist with an onsite coagulation laboratory, ideally associated with a hemophilia treatment center, is integral to the management of CVD in PWH.

The massive tissue factor stimulus results in excess intravascular thrombin, which overcomes the anticoagulant systems and leads to thrombosis. Because of consumption of coagulation factors and platelets, DIC also has a hemorrhagic phase. Treatment of the bleeding patient with DIC is supportive with the use of blood components.

linear over 6 logs (dilutions 10 Ϫ2 through 10 Ϫ7 ). The IC was consistently detected in dilutions 10 Ϫ6 through 10 Ϫ9 . To determine interassay variation, we assayed 7 clinical samples positive for B. pertussis DNA within the linearity range of the assay 5 times on 5 different days (including reextraction each day). The SD calculated from the crossing points obtained was 0.08 -1.09. We determined intraassay variation by assaying 3 B. pertussis-positive clinical samples within the linearity range of the assay 5 times within a single assay (including different extractions). Mean crossing points were calculated, and the SD was 0.14 -1.04. The mean melting peak was at 62°C (range, 61.5-62.5°C).When clinical samples were tested, 46 of 219 nasopharyngeal swabs were found to be positive for B. pertussis DNA, with melting points at the expected temperature. Sera were obtained 4 weeks later from all patients with positive results and tested with the Serion ELISA classic for B. pertussis IgA (Institut Virion\Serion GmbH). A positive IgA result was obtained for all sera from patients with a positive PCR result. The new homologous IC was consistently detected in all negative and in 10 (22%) of 46 positive clinical samples. Extraction of samples was completed within 2 h. After the centrifugation step, real-time PCR took another 55 min. No contamination was observed during the entire study.PCR amplification may fail because of interference from PCR inhibitors; therefore, ICs have been incorporated in molecular assays for detection of B. pertussis (10,13 ). The homologous IC used in this study was coextracted with the clinical samples and coamplified with the same primers used for the target DNA. This procedure ensures accurate control of the entire molecular assay and represents the state of the art for ICs. The B. pertussis-specific IC gave positive results for all negative samples throughout the whole study, indicating successful removal of potential inhibitors by the extraction method. Contrary to a recent study using an identical amplification protocol (13 ), the sensitivity of the assay in this study was not affected by introduction of the homologous IC. Sensitivity to strains other than B. pertussis may also not be affected by introduction of this IC. In 78% of positive samples, competitive inhibition prevented detection of the IC.In conclusion, the newly established assay includes all of the features required for molecular detection of B. pertussis in the routine diagnostic laboratory. This molecular assay is suitable for the routine diagnostic laboratory and allows rapid and safe diagnosis of B. pertussis. Through autoverification, a customized expert system within the Laboratory Information System (LIS), a computer performs the initial review and verification of test results based on a predetermined set of boundaries or rules, as established by the laboratory (1 ). A carefully designed system can be an important tool in addressing such crucial issues as medical errors, test turnaround time (TAT), shortages in perso...

Background: Immunomodulatory strategies in heparin-induced thrombocytopenia (HIT) include the use of intravenous immune globulin (IVIG) and therapeutic plasma exchange (TPE). The optimal application of these therapies is unknown and outcomes data are limited. We investigated treatment categories and laboratory and clinical outcomes of IVIG and/or TPE in HIT with a systematic literature review. Study Design and Methods: We searched MEDLINE, Embase, and Web of Science through December 2019 for studies combining controlled vocabulary and keywords related to thrombocytopenia, heparin, TPE, and IVIG. The primary outcome was treatment indication. Secondary outcomes were platelet recovery, HIT laboratory parameters, heparin re-exposure, and post-treatment course. Case-level data were analyzed by qualitative synthesis. Results: After 4241 references were screened, we identified 60 studies with four main categories of IVIG and/or TPE use as follows: (a) treatment of refractory HIT (n = 35; 31%); (b) initial therapy (n = 45; 40%); (c) cardiopulmonary bypass surgery (CPB; n = 30; 27%); and (d) other (n = 2; 2%). IVIG was most commonly used for the treatment of refractory HIT while TPE was primarily used to facilitate heparin exposure during CPB. Both IVIG and TPE were

These survey results demonstrate wide interinstitutional variation in replacement fluid selection to manage hemostasis in patients undergoing TPE. Further studies are needed to guide optimal hemostasis management with TPE.